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result sql = Biochemical aspects of globoid and metachromatic leukodystrophies.
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char = c;code = 99
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char = p;code = 112
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char = ;code = 32
char = m;code = 109
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char = l;code = 108
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char = d;code = 100
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char = t;code = 116
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char = o;code = 111
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char = d;code = 100
char = ;code = 32
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char = r;code = 114
char = y;code = 121
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char = u;code = 117
char = l;code = 108
char = f;code = 102
char = a;code = 97
char = t;code = 116
char = a;code = 97
char = s;code = 115
char = e;code = 101
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char = A;code = 65
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char = ;code = 32
char = r;code = 114
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char = p;code = 112
char = e;code = 101
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char = ;code = 32
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char = h;code = 104
char = e;code = 101
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char = m;code = 109
char = u;code = 117
char = t;code = 116
char = a;code = 97
char = n;code = 110
char = t;code = 116
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char = e;code = 101
char = n;code = 110
char = z;code = 122
char = y;code = 121
char = m;code = 109
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = h;code = 104
char = a;code = 97
char = v;code = 118
char = e;code = 101
char = ;code = 32
char = d;code = 100
char = i;code = 105
char = f;code = 102
char = f;code = 102
char = e;code = 101
char = r;code = 114
char = e;code = 101
char = n;code = 110
char = t;code = 116
char = ;code = 32
char = k;code = 107
char = i;code = 105
char = n;code = 110
char = e;code = 101
char = t;code = 116
char = i;code = 105
char = c;code = 99
char = ;code = 32
char = p;code = 112
char = r;code = 114
char = o;code = 111
char = p;code = 112
char = e;code = 101
char = r;code = 114
char = t;code = 116
char = i;code = 105
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = c;code = 99
char = o;code = 111
char = m;code = 109
char = p;code = 112
char = a;code = 97
char = r;code = 114
char = e;code = 101
char = d;code = 100
char = ;code = 32
char = t;code = 116
char = o;code = 111
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = e;code = 101
char = ;code = 32
char = e;code = 101
char = n;code = 110
char = z;code = 122
char = y;code = 121
char = m;code = 109
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = f;code = 102
char = r;code = 114
char = o;code = 111
char = m;code = 109
char = ;code = 32
char = n;code = 110
char = o;code = 111
char = r;code = 114
char = m;code = 109
char = a;code = 97
char = l;code = 108
char = ;code = 32
char = s;code = 115
char = u;code = 117
char = b;code = 98
char = j;code = 106
char = e;code = 101
char = c;code = 99
char = t;code = 116
char = s;code = 115
char = .;code = 46
char = ;code = 32
char = H;code = 72
char = o;code = 111
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char = e;code = 101
char = v;code = 118
char = e;code = 101
char = r;code = 114
char = ,;code = 44
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char = n;code = 110
char = ;code = 32
char = c;code = 99
char = r;code = 114
char = o;code = 111
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char = s;code = 115
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char = r;code = 114
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char = a;code = 97
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char = t;code = 116
char = h;code = 104
char = ;code = 32
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char = e;code = 101
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char = ;code = 32
char = t;code = 116
char = o;code = 111
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = e;code = 101
char = s;code = 115
char = e;code = 101
char = ;code = 32
char = e;code = 101
char = n;code = 110
char = z;code = 122
char = y;code = 121
char = m;code = 109
char = e;code = 101
char = s;code = 115
char = .;code = 46
char = ;code = 32
char = S;code = 83
char = i;code = 105
char = n;code = 110
char = c;code = 99
char = e;code = 101
char = ;code = 32
char = p;code = 112
char = a;code = 97
char = r;code = 114
char = t;code = 116
char = i;code = 105
char = a;code = 97
char = l;code = 108
char = l;code = 108
char = y;code = 121
char = ;code = 32
char = p;code = 112
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char = r;code = 114
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char = f;code = 102
char = i;code = 105
char = e;code = 101
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char = o;code = 111
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char = e;code = 101
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char = l;code = 108
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char = a;code = 97
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char = l;code = 108
char = a;code = 97
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char = l;code = 108
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char = ;code = 32
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char = l;code = 108
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char = o;code = 111
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char = a;code = 97
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char = d;code = 100
char = ;code = 32
char = m;code = 109
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char = c;code = 99
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char = r;code = 114
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char = m;code = 109
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char = i;code = 105
char = c;code = 99
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char = l;code = 108
char = e;code = 101
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char = k;code = 107
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char = y;code = 121
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char = t;code = 116
char = r;code = 114
char = o;code = 111
char = p;code = 112
char = h;code = 104
char = i;code = 105
char = e;code = 101
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char = ;code = 32
char = i;code = 105
char = s;code = 115
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char = d;code = 100
char = i;code = 105
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char = c;code = 99
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char = s;code = 115
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char = e;code = 101
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char = <;code = 60
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char = >;code = 62
result sql = Galactosylceramides and sulfogalactosylceramides are characteristic lipids of the myelin sheath. Two genetically determined leukodystrophies are caused by an inability to enzymically hydrolyze these glycolipids. Thus, a deficiency of galactocerebroside beta-galactosidase results in globoid cell leukodystrophy, whereas a reduced activity of arylsulfatase A is responsible for metachromatic leukodystrophy. Besides these disorders, deficiencies of arylsulfatases A, B, C, and other sulfatases have been shown in a distinct condition called "multiple sulfatase deficiency." All of these disorders are fatal and are characterized by marked demyelination and severe mental retardation. The cause of this demyelination is not known. However, cytotoxic galactosylsphingosine and sulfogalactosylsphingosine have been suggested as the agents responsible for this demyelination. Recent immunological studies have also shown that patients with globoid and metachromatic leukodystrophies contain a mutant galactocerebroside beta-galactosidase and arylsulfatase A, respectively. The mutant enzymes have different kinetic properties compared to the enzymes from normal subjects. However, they can cross-react with antibodies to these enzymes. Since partially purified preparations of galactocerebroside beta-galactosidase and homogeneous arylsulfatase A are now available, the possibility of enzyme replacement therapy in globoid and metachromatic leukodystrophies is discussed.
char = i;code = 105
char = o;code = 111
char = c;code = 99
char = h;code = 104
char = e;code = 101
char = m;code = 109
char = i;code = 105
char = c;code = 99
char = a;code = 97
char = l;code = 108
char = ;code = 32
char = a;code = 97
char = s;code = 115
char = p;code = 112
char = e;code = 101
char = c;code = 99
char = t;code = 116
char = s;code = 115
char = ;code = 32
char = o;code = 111
char = f;code = 102
char = ;code = 32
char = g;code = 103
char = l;code = 108
char = o;code = 111
char = b;code = 98
char = o;code = 111
char = i;code = 105
char = d;code = 100
char = ;code = 32
char = a;code = 97
char = n;code = 110
char = d;code = 100
char = ;code = 32
char = m;code = 109
char = e;code = 101
char = t;code = 116
char = a;code = 97
char = c;code = 99
char = h;code = 104
char = r;code = 114
char = o;code = 111
char = m;code = 109
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = c;code = 99
char = ;code = 32
char = l;code = 108
char = e;code = 101
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char = k;code = 107
char = o;code = 111
char = d;code = 100
char = y;code = 121
char = s;code = 115
char = t;code = 116
char = r;code = 114
char = o;code = 111
char = p;code = 112
char = h;code = 104
char = i;code = 105
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char = s;code = 115
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result sql = Biochemical aspects of globoid and metachromatic leukodystrophies.
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char = y;code = 121
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char = e;code = 101
char = r;code = 114
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char = i;code = 105
char = d;code = 100
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char = s;code = 115
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char = a;code = 97
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char = h;code = 104
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char = a;code = 97
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char = t;code = 116
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char = h;code = 104
char = .;code = 46
char = ;code = 32
char = T;code = 84
char = w;code = 119
char = o;code = 111
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char = g;code = 103
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char = e;code = 101
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char = i;code = 105
char = c;code = 99
char = a;code = 97
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char = y;code = 121
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char = t;code = 116
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char = e;code = 101
char = ;code = 32
char = g;code = 103
char = l;code = 108
char = y;code = 121
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char = o;code = 111
char = l;code = 108
char = i;code = 105
char = p;code = 112
char = i;code = 105
char = d;code = 100
char = s;code = 115
char = .;code = 46
char = ;code = 32
char = T;code = 84
char = h;code = 104
char = u;code = 117
char = s;code = 115
char = ,;code = 44
char = ;code = 32
char = a;code = 97
char = ;code = 32
char = d;code = 100
char = e;code = 101
char = f;code = 102
char = i;code = 105
char = c;code = 99
char = i;code = 105
char = e;code = 101
char = n;code = 110
char = c;code = 99
char = y;code = 121
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char = o;code = 111
char = f;code = 102
char = ;code = 32
char = g;code = 103
char = a;code = 97
char = l;code = 108
char = a;code = 97
char = c;code = 99
char = t;code = 116
char = o;code = 111
char = c;code = 99
char = e;code = 101
char = r;code = 114
char = e;code = 101
char = b;code = 98
char = r;code = 114
char = o;code = 111
char = s;code = 115
char = i;code = 105
char = d;code = 100
char = e;code = 101
char = ;code = 32
char = b;code = 98
char = e;code = 101
char = t;code = 116
char = a;code = 97
char = -;code = 45
char = g;code = 103
char = a;code = 97
char = l;code = 108
char = a;code = 97
char = c;code = 99
char = t;code = 116
char = o;code = 111
char = s;code = 115
char = i;code = 105
char = d;code = 100
char = a;code = 97
char = s;code = 115
char = e;code = 101
char = ;code = 32
char = r;code = 114
char = e;code = 101
char = s;code = 115
char = u;code = 117
char = l;code = 108
char = t;code = 116
char = s;code = 115
char = ;code = 32
char = i;code = 105
char = n;code = 110
char = ;code = 32
char = g;code = 103
char = l;code = 108
char = o;code = 111
char = b;code = 98
char = o;code = 111
char = i;code = 105
char = d;code = 100
char = ;code = 32
char = c;code = 99
char = e;code = 101
char = l;code = 108
char = l;code = 108
char = ;code = 32
char = l;code = 108
char = e;code = 101
char = u;code = 117
char = k;code = 107
char = o;code = 111
char = d;code = 100
char = y;code = 121
char = s;code = 115
char = t;code = 116
char = r;code = 114
char = o;code = 111
char = p;code = 112
char = h;code = 104
char = y;code = 121
char = ,;code = 44
char = ;code = 32
char = w;code = 119
char = h;code = 104
char = e;code = 101
char = r;code = 114
char = e;code = 101
char = a;code = 97
char = s;code = 115
char = ;code = 32
char = a;code = 97
char = ;code = 32
char = r;code = 114
char = e;code = 101
char = d;code = 100
char = u;code = 117
char = c;code = 99
char = e;code = 101
char = d;code = 100
char = ;code = 32
char = a;code = 97
char = c;code = 99
char = t;code = 116
char = i;code = 105
char = v;code = 118
char = i;code = 105
char = t;code = 116
char = y;code = 121
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char = o;code = 111
char = f;code = 102
char = ;code = 32
char = a;code = 97
char = r;code = 114
char = y;code = 121
char = l;code = 108
char = s;code = 115
char = u;code = 117
char = l;code = 108
char = f;code = 102
char = a;code = 97
char = t;code = 116
char = a;code = 97
char = s;code = 115
char = e;code = 101
char = ;code = 32
char = A;code = 65
char = ;code = 32
char = i;code = 105
char = s;code = 115
char = ;code = 32
char = r;code = 114
char = e;code = 101
char = s;code = 115
char = p;code = 112
char = o;code = 111
char = n;code = 110
char = s;code = 115
char = i;code = 105
char = b;code = 98
char = l;code = 108
char = e;code = 101
char = ;code = 32
char = f;code = 102
char = o;code = 111
char = r;code = 114
char = ;code = 32
char = m;code = 109
char = e;code = 101
char = t;code = 116
char = a;code = 97
char = c;code = 99
char = h;code = 104
char = r;code = 114
char = o;code = 111
char = m;code = 109
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = c;code = 99
char = ;code = 32
char = l;code = 108
char = e;code = 101
char = u;code = 117
char = k;code = 107
char = o;code = 111
char = d;code = 100
char = y;code = 121
char = s;code = 115
char = t;code = 116
char = r;code = 114
char = o;code = 111
char = p;code = 112
char = h;code = 104
char = y;code = 121
char = .;code = 46
char = ;code = 32
char = B;code = 66
char = e;code = 101
char = s;code = 115
char = i;code = 105
char = d;code = 100
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = e;code = 101
char = s;code = 115
char = e;code = 101
char = ;code = 32
char = d;code = 100
char = i;code = 105
char = s;code = 115
char = o;code = 111
char = r;code = 114
char = d;code = 100
char = e;code = 101
char = r;code = 114
char = s;code = 115
char = ,;code = 44
char = ;code = 32
char = d;code = 100
char = e;code = 101
char = f;code = 102
char = i;code = 105
char = c;code = 99
char = i;code = 105
char = e;code = 101
char = n;code = 110
char = c;code = 99
char = i;code = 105
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = o;code = 111
char = f;code = 102
char = ;code = 32
char = a;code = 97
char = r;code = 114
char = y;code = 121
char = l;code = 108
char = s;code = 115
char = u;code = 117
char = l;code = 108
char = f;code = 102
char = a;code = 97
char = t;code = 116
char = a;code = 97
char = s;code = 115
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = A;code = 65
char = ,;code = 44
char = ;code = 32
char = B;code = 66
char = ,;code = 44
char = ;code = 32
char = C;code = 67
char = ,;code = 44
char = ;code = 32
char = a;code = 97
char = n;code = 110
char = d;code = 100
char = ;code = 32
char = o;code = 111
char = t;code = 116
char = h;code = 104
char = e;code = 101
char = r;code = 114
char = ;code = 32
char = s;code = 115
char = u;code = 117
char = l;code = 108
char = f;code = 102
char = a;code = 97
char = t;code = 116
char = a;code = 97
char = s;code = 115
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = h;code = 104
char = a;code = 97
char = v;code = 118
char = e;code = 101
char = ;code = 32
char = b;code = 98
char = e;code = 101
char = e;code = 101
char = n;code = 110
char = ;code = 32
char = s;code = 115
char = h;code = 104
char = o;code = 111
char = w;code = 119
char = n;code = 110
char = ;code = 32
char = i;code = 105
char = n;code = 110
char = ;code = 32
char = a;code = 97
char = ;code = 32
char = d;code = 100
char = i;code = 105
char = s;code = 115
char = t;code = 116
char = i;code = 105
char = n;code = 110
char = c;code = 99
char = t;code = 116
char = ;code = 32
char = c;code = 99
char = o;code = 111
char = n;code = 110
char = d;code = 100
char = i;code = 105
char = t;code = 116
char = i;code = 105
char = o;code = 111
char = n;code = 110
char = ;code = 32
char = c;code = 99
char = a;code = 97
char = l;code = 108
char = l;code = 108
char = e;code = 101
char = d;code = 100
char = ;code = 32
char = ";code = 34
char = m;code = 109
char = u;code = 117
char = l;code = 108
char = t;code = 116
char = i;code = 105
char = p;code = 112
char = l;code = 108
char = e;code = 101
char = ;code = 32
char = s;code = 115
char = u;code = 117
char = l;code = 108
char = f;code = 102
char = a;code = 97
char = t;code = 116
char = a;code = 97
char = s;code = 115
char = e;code = 101
char = ;code = 32
char = d;code = 100
char = e;code = 101
char = f;code = 102
char = i;code = 105
char = c;code = 99
char = i;code = 105
char = e;code = 101
char = n;code = 110
char = c;code = 99
char = y;code = 121
char = .;code = 46
char = ";code = 34
char = ;code = 32
char = A;code = 65
char = l;code = 108
char = l;code = 108
char = ;code = 32
char = o;code = 111
char = f;code = 102
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = e;code = 101
char = s;code = 115
char = e;code = 101
char = ;code = 32
char = d;code = 100
char = i;code = 105
char = s;code = 115
char = o;code = 111
char = r;code = 114
char = d;code = 100
char = e;code = 101
char = r;code = 114
char = s;code = 115
char = ;code = 32
char = a;code = 97
char = r;code = 114
char = e;code = 101
char = ;code = 32
char = f;code = 102
char = a;code = 97
char = t;code = 116
char = a;code = 97
char = l;code = 108
char = ;code = 32
char = a;code = 97
char = n;code = 110
char = d;code = 100
char = ;code = 32
char = a;code = 97
char = r;code = 114
char = e;code = 101
char = ;code = 32
char = c;code = 99
char = h;code = 104
char = a;code = 97
char = r;code = 114
char = a;code = 97
char = c;code = 99
char = t;code = 116
char = e;code = 101
char = r;code = 114
char = i;code = 105
char = z;code = 122
char = e;code = 101
char = d;code = 100
char = ;code = 32
char = b;code = 98
char = y;code = 121
char = ;code = 32
char = m;code = 109
char = a;code = 97
char = r;code = 114
char = k;code = 107
char = e;code = 101
char = d;code = 100
char = ;code = 32
char = d;code = 100
char = e;code = 101
char = m;code = 109
char = y;code = 121
char = e;code = 101
char = l;code = 108
char = i;code = 105
char = n;code = 110
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = o;code = 111
char = n;code = 110
char = ;code = 32
char = a;code = 97
char = n;code = 110
char = d;code = 100
char = ;code = 32
char = s;code = 115
char = e;code = 101
char = v;code = 118
char = e;code = 101
char = r;code = 114
char = e;code = 101
char = ;code = 32
char = m;code = 109
char = e;code = 101
char = n;code = 110
char = t;code = 116
char = a;code = 97
char = l;code = 108
char = ;code = 32
char = r;code = 114
char = e;code = 101
char = t;code = 116
char = a;code = 97
char = r;code = 114
char = d;code = 100
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = o;code = 111
char = n;code = 110
char = .;code = 46
char = ;code = 32
char = T;code = 84
char = h;code = 104
char = e;code = 101
char = ;code = 32
char = c;code = 99
char = a;code = 97
char = u;code = 117
char = s;code = 115
char = e;code = 101
char = ;code = 32
char = o;code = 111
char = f;code = 102
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = i;code = 105
char = s;code = 115
char = ;code = 32
char = d;code = 100
char = e;code = 101
char = m;code = 109
char = y;code = 121
char = e;code = 101
char = l;code = 108
char = i;code = 105
char = n;code = 110
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = o;code = 111
char = n;code = 110
char = ;code = 32
char = i;code = 105
char = s;code = 115
char = ;code = 32
char = n;code = 110
char = o;code = 111
char = t;code = 116
char = ;code = 32
char = k;code = 107
char = n;code = 110
char = o;code = 111
char = w;code = 119
char = n;code = 110
char = .;code = 46
char = ;code = 32
char = H;code = 72
char = o;code = 111
char = w;code = 119
char = e;code = 101
char = v;code = 118
char = e;code = 101
char = r;code = 114
char = ,;code = 44
char = ;code = 32
char = c;code = 99
char = y;code = 121
char = t;code = 116
char = o;code = 111
char = t;code = 116
char = o;code = 111
char = x;code = 120
char = i;code = 105
char = c;code = 99
char = ;code = 32
char = g;code = 103
char = a;code = 97
char = l;code = 108
char = a;code = 97
char = c;code = 99
char = t;code = 116
char = o;code = 111
char = s;code = 115
char = y;code = 121
char = l;code = 108
char = s;code = 115
char = p;code = 112
char = h;code = 104
char = i;code = 105
char = n;code = 110
char = g;code = 103
char = o;code = 111
char = s;code = 115
char = i;code = 105
char = n;code = 110
char = e;code = 101
char = ;code = 32
char = a;code = 97
char = n;code = 110
char = d;code = 100
char = ;code = 32
char = s;code = 115
char = u;code = 117
char = l;code = 108
char = f;code = 102
char = o;code = 111
char = g;code = 103
char = a;code = 97
char = l;code = 108
char = a;code = 97
char = c;code = 99
char = t;code = 116
char = o;code = 111
char = s;code = 115
char = y;code = 121
char = l;code = 108
char = s;code = 115
char = p;code = 112
char = h;code = 104
char = i;code = 105
char = n;code = 110
char = g;code = 103
char = o;code = 111
char = s;code = 115
char = i;code = 105
char = n;code = 110
char = e;code = 101
char = ;code = 32
char = h;code = 104
char = a;code = 97
char = v;code = 118
char = e;code = 101
char = ;code = 32
char = b;code = 98
char = e;code = 101
char = e;code = 101
char = n;code = 110
char = ;code = 32
char = s;code = 115
char = u;code = 117
char = g;code = 103
char = g;code = 103
char = e;code = 101
char = s;code = 115
char = t;code = 116
char = e;code = 101
char = d;code = 100
char = ;code = 32
char = a;code = 97
char = s;code = 115
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = e;code = 101
char = ;code = 32
char = a;code = 97
char = g;code = 103
char = e;code = 101
char = n;code = 110
char = t;code = 116
char = s;code = 115
char = ;code = 32
char = r;code = 114
char = e;code = 101
char = s;code = 115
char = p;code = 112
char = o;code = 111
char = n;code = 110
char = s;code = 115
char = i;code = 105
char = b;code = 98
char = l;code = 108
char = e;code = 101
char = ;code = 32
char = f;code = 102
char = o;code = 111
char = r;code = 114
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = i;code = 105
char = s;code = 115
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char = d;code = 100
char = e;code = 101
char = m;code = 109
char = y;code = 121
char = e;code = 101
char = l;code = 108
char = i;code = 105
char = n;code = 110
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = o;code = 111
char = n;code = 110
char = .;code = 46
char = ;code = 32
char = R;code = 82
char = e;code = 101
char = c;code = 99
char = e;code = 101
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char = t;code = 116
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char = i;code = 105
char = m;code = 109
char = m;code = 109
char = u;code = 117
char = n;code = 110
char = o;code = 111
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char = o;code = 111
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char = i;code = 105
char = c;code = 99
char = a;code = 97
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char = ;code = 32
char = s;code = 115
char = t;code = 116
char = u;code = 117
char = d;code = 100
char = i;code = 105
char = e;code = 101
char = s;code = 115
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char = a;code = 97
char = v;code = 118
char = e;code = 101
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char = a;code = 97
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char = s;code = 115
char = o;code = 111
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char = s;code = 115
char = h;code = 104
char = o;code = 111
char = w;code = 119
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char = t;code = 116
char = h;code = 104
char = a;code = 97
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char = p;code = 112
char = a;code = 97
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char = i;code = 105
char = e;code = 101
char = n;code = 110
char = t;code = 116
char = s;code = 115
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char = w;code = 119
char = i;code = 105
char = t;code = 116
char = h;code = 104
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char = g;code = 103
char = l;code = 108
char = o;code = 111
char = b;code = 98
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char = d;code = 100
char = ;code = 32
char = a;code = 97
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char = d;code = 100
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char = m;code = 109
char = e;code = 101
char = t;code = 116
char = a;code = 97
char = c;code = 99
char = h;code = 104
char = r;code = 114
char = o;code = 111
char = m;code = 109
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = c;code = 99
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char = l;code = 108
char = e;code = 101
char = u;code = 117
char = k;code = 107
char = o;code = 111
char = d;code = 100
char = y;code = 121
char = s;code = 115
char = t;code = 116
char = r;code = 114
char = o;code = 111
char = p;code = 112
char = h;code = 104
char = i;code = 105
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = c;code = 99
char = o;code = 111
char = n;code = 110
char = t;code = 116
char = a;code = 97
char = i;code = 105
char = n;code = 110
char = ;code = 32
char = a;code = 97
char = ;code = 32
char = m;code = 109
char = u;code = 117
char = t;code = 116
char = a;code = 97
char = n;code = 110
char = t;code = 116
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char = g;code = 103
char = a;code = 97
char = l;code = 108
char = a;code = 97
char = c;code = 99
char = t;code = 116
char = o;code = 111
char = c;code = 99
char = e;code = 101
char = r;code = 114
char = e;code = 101
char = b;code = 98
char = r;code = 114
char = o;code = 111
char = s;code = 115
char = i;code = 105
char = d;code = 100
char = e;code = 101
char = ;code = 32
char = b;code = 98
char = e;code = 101
char = t;code = 116
char = a;code = 97
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char = g;code = 103
char = a;code = 97
char = l;code = 108
char = a;code = 97
char = c;code = 99
char = t;code = 116
char = o;code = 111
char = s;code = 115
char = i;code = 105
char = d;code = 100
char = a;code = 97
char = s;code = 115
char = e;code = 101
char = ;code = 32
char = a;code = 97
char = n;code = 110
char = d;code = 100
char = ;code = 32
char = a;code = 97
char = r;code = 114
char = y;code = 121
char = l;code = 108
char = s;code = 115
char = u;code = 117
char = l;code = 108
char = f;code = 102
char = a;code = 97
char = t;code = 116
char = a;code = 97
char = s;code = 115
char = e;code = 101
char = ;code = 32
char = A;code = 65
char = ,;code = 44
char = ;code = 32
char = r;code = 114
char = e;code = 101
char = s;code = 115
char = p;code = 112
char = e;code = 101
char = c;code = 99
char = t;code = 116
char = i;code = 105
char = v;code = 118
char = e;code = 101
char = l;code = 108
char = y;code = 121
char = .;code = 46
char = ;code = 32
char = T;code = 84
char = h;code = 104
char = e;code = 101
char = ;code = 32
char = m;code = 109
char = u;code = 117
char = t;code = 116
char = a;code = 97
char = n;code = 110
char = t;code = 116
char = ;code = 32
char = e;code = 101
char = n;code = 110
char = z;code = 122
char = y;code = 121
char = m;code = 109
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = h;code = 104
char = a;code = 97
char = v;code = 118
char = e;code = 101
char = ;code = 32
char = d;code = 100
char = i;code = 105
char = f;code = 102
char = f;code = 102
char = e;code = 101
char = r;code = 114
char = e;code = 101
char = n;code = 110
char = t;code = 116
char = ;code = 32
char = k;code = 107
char = i;code = 105
char = n;code = 110
char = e;code = 101
char = t;code = 116
char = i;code = 105
char = c;code = 99
char = ;code = 32
char = p;code = 112
char = r;code = 114
char = o;code = 111
char = p;code = 112
char = e;code = 101
char = r;code = 114
char = t;code = 116
char = i;code = 105
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = c;code = 99
char = o;code = 111
char = m;code = 109
char = p;code = 112
char = a;code = 97
char = r;code = 114
char = e;code = 101
char = d;code = 100
char = ;code = 32
char = t;code = 116
char = o;code = 111
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = e;code = 101
char = ;code = 32
char = e;code = 101
char = n;code = 110
char = z;code = 122
char = y;code = 121
char = m;code = 109
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = f;code = 102
char = r;code = 114
char = o;code = 111
char = m;code = 109
char = ;code = 32
char = n;code = 110
char = o;code = 111
char = r;code = 114
char = m;code = 109
char = a;code = 97
char = l;code = 108
char = ;code = 32
char = s;code = 115
char = u;code = 117
char = b;code = 98
char = j;code = 106
char = e;code = 101
char = c;code = 99
char = t;code = 116
char = s;code = 115
char = .;code = 46
char = ;code = 32
char = H;code = 72
char = o;code = 111
char = w;code = 119
char = e;code = 101
char = v;code = 118
char = e;code = 101
char = r;code = 114
char = ,;code = 44
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = e;code = 101
char = y;code = 121
char = ;code = 32
char = c;code = 99
char = a;code = 97
char = n;code = 110
char = ;code = 32
char = c;code = 99
char = r;code = 114
char = o;code = 111
char = s;code = 115
char = s;code = 115
char = -;code = 45
char = r;code = 114
char = e;code = 101
char = a;code = 97
char = c;code = 99
char = t;code = 116
char = ;code = 32
char = w;code = 119
char = i;code = 105
char = t;code = 116
char = h;code = 104
char = ;code = 32
char = a;code = 97
char = n;code = 110
char = t;code = 116
char = i;code = 105
char = b;code = 98
char = o;code = 111
char = d;code = 100
char = i;code = 105
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = t;code = 116
char = o;code = 111
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = e;code = 101
char = s;code = 115
char = e;code = 101
char = ;code = 32
char = e;code = 101
char = n;code = 110
char = z;code = 122
char = y;code = 121
char = m;code = 109
char = e;code = 101
char = s;code = 115
char = .;code = 46
char = ;code = 32
char = S;code = 83
char = i;code = 105
char = n;code = 110
char = c;code = 99
char = e;code = 101
char = ;code = 32
char = p;code = 112
char = a;code = 97
char = r;code = 114
char = t;code = 116
char = i;code = 105
char = a;code = 97
char = l;code = 108
char = l;code = 108
char = y;code = 121
char = ;code = 32
char = p;code = 112
char = u;code = 117
char = r;code = 114
char = i;code = 105
char = f;code = 102
char = i;code = 105
char = e;code = 101
char = d;code = 100
char = ;code = 32
char = p;code = 112
char = r;code = 114
char = e;code = 101
char = p;code = 112
char = a;code = 97
char = r;code = 114
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = o;code = 111
char = n;code = 110
char = s;code = 115
char = ;code = 32
char = o;code = 111
char = f;code = 102
char = ;code = 32
char = g;code = 103
char = a;code = 97
char = l;code = 108
char = a;code = 97
char = c;code = 99
char = t;code = 116
char = o;code = 111
char = c;code = 99
char = e;code = 101
char = r;code = 114
char = e;code = 101
char = b;code = 98
char = r;code = 114
char = o;code = 111
char = s;code = 115
char = i;code = 105
char = d;code = 100
char = e;code = 101
char = ;code = 32
char = b;code = 98
char = e;code = 101
char = t;code = 116
char = a;code = 97
char = -;code = 45
char = g;code = 103
char = a;code = 97
char = l;code = 108
char = a;code = 97
char = c;code = 99
char = t;code = 116
char = o;code = 111
char = s;code = 115
char = i;code = 105
char = d;code = 100
char = a;code = 97
char = s;code = 115
char = e;code = 101
char = ;code = 32
char = a;code = 97
char = n;code = 110
char = d;code = 100
char = ;code = 32
char = h;code = 104
char = o;code = 111
char = m;code = 109
char = o;code = 111
char = g;code = 103
char = e;code = 101
char = n;code = 110
char = e;code = 101
char = o;code = 111
char = u;code = 117
char = s;code = 115
char = ;code = 32
char = a;code = 97
char = r;code = 114
char = y;code = 121
char = l;code = 108
char = s;code = 115
char = u;code = 117
char = l;code = 108
char = f;code = 102
char = a;code = 97
char = t;code = 116
char = a;code = 97
char = s;code = 115
char = e;code = 101
char = ;code = 32
char = A;code = 65
char = ;code = 32
char = a;code = 97
char = r;code = 114
char = e;code = 101
char = ;code = 32
char = n;code = 110
char = o;code = 111
char = w;code = 119
char = ;code = 32
char = a;code = 97
char = v;code = 118
char = a;code = 97
char = i;code = 105
char = l;code = 108
char = a;code = 97
char = b;code = 98
char = l;code = 108
char = e;code = 101
char = ,;code = 44
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = e;code = 101
char = ;code = 32
char = p;code = 112
char = o;code = 111
char = s;code = 115
char = s;code = 115
char = i;code = 105
char = b;code = 98
char = i;code = 105
char = l;code = 108
char = i;code = 105
char = t;code = 116
char = y;code = 121
char = ;code = 32
char = o;code = 111
char = f;code = 102
char = ;code = 32
char = e;code = 101
char = n;code = 110
char = z;code = 122
char = y;code = 121
char = m;code = 109
char = e;code = 101
char = ;code = 32
char = r;code = 114
char = e;code = 101
char = p;code = 112
char = l;code = 108
char = a;code = 97
char = c;code = 99
char = e;code = 101
char = m;code = 109
char = e;code = 101
char = n;code = 110
char = t;code = 116
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = e;code = 101
char = r;code = 114
char = a;code = 97
char = p;code = 112
char = y;code = 121
char = ;code = 32
char = i;code = 105
char = n;code = 110
char = ;code = 32
char = g;code = 103
char = l;code = 108
char = o;code = 111
char = b;code = 98
char = o;code = 111
char = i;code = 105
char = d;code = 100
char = ;code = 32
char = a;code = 97
char = n;code = 110
char = d;code = 100
char = ;code = 32
char = m;code = 109
char = e;code = 101
char = t;code = 116
char = a;code = 97
char = c;code = 99
char = h;code = 104
char = r;code = 114
char = o;code = 111
char = m;code = 109
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = c;code = 99
char = ;code = 32
char = l;code = 108
char = e;code = 101
char = u;code = 117
char = k;code = 107
char = o;code = 111
char = d;code = 100
char = y;code = 121
char = s;code = 115
char = t;code = 116
char = r;code = 114
char = o;code = 111
char = p;code = 112
char = h;code = 104
char = i;code = 105
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = i;code = 105
char = s;code = 115
char = ;code = 32
char = d;code = 100
char = i;code = 105
char = s;code = 115
char = c;code = 99
char = u;code = 117
char = s;code = 115
char = s;code = 115
char = e;code = 101
char = d;code = 100
char = .;code = 46
char = <;code = 60
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char = >;code = 62
result sql = Galactosylceramides and sulfogalactosylceramides are characteristic lipids of the myelin sheath. Two genetically determined leukodystrophies are caused by an inability to enzymically hydrolyze these glycolipids. Thus, a deficiency of galactocerebroside beta-galactosidase results in globoid cell leukodystrophy, whereas a reduced activity of arylsulfatase A is responsible for metachromatic leukodystrophy. Besides these disorders, deficiencies of arylsulfatases A, B, C, and other sulfatases have been shown in a distinct condition called "multiple sulfatase deficiency." All of these disorders are fatal and are characterized by marked demyelination and severe mental retardation. The cause of this demyelination is not known. However, cytotoxic galactosylsphingosine and sulfogalactosylsphingosine have been suggested as the agents responsible for this demyelination. Recent immunological studies have also shown that patients with globoid and metachromatic leukodystrophies contain a mutant galactocerebroside beta-galactosidase and arylsulfatase A, respectively. The mutant enzymes have different kinetic properties compared to the enzymes from normal subjects. However, they can cross-react with antibodies to these enzymes. Since partially purified preparations of galactocerebroside beta-galactosidase and homogeneous arylsulfatase A are now available, the possibility of enzyme replacement therapy in globoid and metachromatic leukodystrophies is discussed.
Publication: Neurochem Pathol 1984 Fall ;2(3)::189-218.
Publication Date: 1984 Fall
Study Author(s): Farooqui, A A;Horrocks, L A;
Institution:
Shortcut link to this study:
Publication Date: 1984 Fall
Study Author(s): Farooqui, A A;Horrocks, L A;
Institution:
Shortcut link to this study:
Galactosylceramides and sulfogalactosylceramides are characteristic lipids of the myelin sheath. Two genetically determined leukodystrophies are caused by an inability to enzymically hydrolyze these glycolipids. Thus, a deficiency of galactocerebroside beta-galactosidase results in globoid cell leukodystrophy, whereas a reduced activity of arylsulfatase A is responsible for metachromatic leukodystrophy. Besides these disorders, deficiencies of arylsulfatases A, B, C, and other sulfatases have been shown in a distinct condition called "multiple sulfatase deficiency." All of these disorders are fatal and are characterized by marked demyelination and severe mental retardation. The cause of this demyelination is not known. However, cytotoxic galactosylsphingosine and sulfogalactosylsphingosine have been suggested as the agents responsible for this demyelination. Recent immunological studies have also shown that patients with globoid and metachromatic leukodystrophies contain a mutant galactocerebroside beta-galactosidase and arylsulfatase A, respectively. The mutant enzymes have different kinetic properties compared to the enzymes from normal subjects. However, they can cross-react with antibodies to these enzymes. Since partially purified preparations of galactocerebroside beta-galactosidase and homogeneous arylsulfatase A are now available, the possibility of enzyme replacement therapy in globoid and metachromatic leukodystrophies is discussed.
PMID: 6152665
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