|
|
char = [;code = 91
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result sql = [Inheritance in erythropoietic protoporphyria].
[Inheritance in erythropoietic protoporphyria].char = E;code = 69
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char = g;code = 103
char = e;code = 101
char = s;code = 115
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char = s;code = 115
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char = t;code = 116
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char = ;code = 32
char = t;code = 116
char = h;code = 104
char = e;code = 101
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char = I;code = 73
char = V;code = 86
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char = 4;code = 52
char = 8;code = 56
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char = l;code = 108
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char = e;code = 101
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char = m;code = 109
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char = t;code = 116
char = i;code = 105
char = o;code = 111
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char = a;code = 97
char = l;code = 108
char = ;code = 32
char = e;code = 101
char = v;code = 118
char = e;code = 101
char = n;code = 110
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char = ;code = 32
char = t;code = 116
char = h;code = 104
char = a;code = 97
char = t;code = 116
char = ;code = 32
char = o;code = 111
char = c;code = 99
char = c;code = 99
char = u;code = 117
char = r;code = 114
char = r;code = 114
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char = d;code = 100
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char = 6;code = 54
char = 0;code = 48
char = ;code = 32
char = K;code = 75
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char = r;code = 114
char = s;code = 115
char = ;code = 32
char = a;code = 97
char = g;code = 103
char = o;code = 111
char = .;code = 46
char = ;code = 32
char = A;code = 65
char = c;code = 99
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char = i;code = 105
char = r;code = 114
char = e;code = 101
char = d;code = 100
char = ;code = 32
char = s;code = 115
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 = m;code = 109
char = u;code = 117
char = t;code = 116
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = o;code = 111
char = n;code = 110
char = ;code = 32
char = o;code = 111
char = f;code = 102
char = ;code = 32
char = F;code = 70
char = E;code = 69
char = C;code = 67
char = H;code = 72
char = ;code = 32
char = s;code = 115
char = e;code = 101
char = c;code = 99
char = o;code = 111
char = n;code = 110
char = d;code = 100
char = a;code = 97
char = r;code = 114
char = y;code = 121
char = ;code = 32
char = t;code = 116
char = o;code = 111
char = ;code = 32
char = m;code = 109
char = y;code = 121
char = e;code = 101
char = l;code = 108
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char = d;code = 100
char = ;code = 32
char = d;code = 100
char = i;code = 105
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char = e;code = 101
char = a;code = 97
char = s;code = 115
char = e;code = 101
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char = m;code = 109
char = a;code = 97
char = y;code = 121
char = ;code = 32
char = a;code = 97
char = l;code = 108
char = s;code = 115
char = o;code = 111
char = ;code = 32
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char = x;code = 120
char = c;code = 99
char = e;code = 101
char = p;code = 112
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char = a;code = 97
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char = l;code = 108
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char = ;code = 32
char = c;code = 99
char = a;code = 97
char = u;code = 117
char = s;code = 115
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char = ;code = 32
char = E;code = 69
char = P;code = 80
char = P;code = 80
char = ;code = 32
char = (;code = 40
char = <;code = 60
char = 1;code = 49
char = %;code = 37
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char = .;code = 46
char = ;code = 32
char = F;code = 70
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char = a;code = 97
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char = l;code = 108
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char = P;code = 80
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char = h;code = 104
char = a;code = 97
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char = e;code = 101
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char = X;code = 88
char = -;code = 45
char = l;code = 108
char = i;code = 105
char = n;code = 110
char = k;code = 107
char = e;code = 101
char = d;code = 100
char = ;code = 32
char = d;code = 100
char = o;code = 111
char = m;code = 109
char = i;code = 105
char = n;code = 110
char = a;code = 97
char = n;code = 110
char = t;code = 116
char = ;code = 32
char = p;code = 112
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char = o;code = 111
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char = o;code = 111
char = p;code = 112
char = o;code = 111
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char = p;code = 112
char = h;code = 104
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char = ;code = 32
char = (;code = 40
char = M;code = 77
char = I;code = 73
char = M;code = 77
char = ;code = 32
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char = 0;code = 48
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char = -;code = 45
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char = c;code = 99
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char = u;code = 117
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char = o;code = 111
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char = i;code = 105
char = n;code = 110
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = e;code = 101
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char = A;code = 65
char = S;code = 83
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char = r;code = 114
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char = a;code = 97
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char = e;code = 101
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char = o;code = 111
char = f;code = 102
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char = o;code = 111
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char = o;code = 111
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char = r;code = 114
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char = w;code = 119
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char = H;code = 72
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char = <;code = 60
char = b;code = 98
char = r;code = 114
char = /;code = 47
char = >;code = 62
result sql = Erythropoietic protoporphyria (EPP) is an inherited disorder of heme biosynthesis that results from an accumulation of protoporphyrin IX in erythroid cells, plasma, skin and liver. EPP leads to acute photosensitivity and, in about 2% of patients, liver disease. EPP is a complex syndrome in which two genes are independently involved: FECH and ALAS2. More than 96% of unrelated EPP patients have ferrochelatase (FECH) deficiency (MIM 177000). Four percent of them present with autosomal recessive inheritance with two mutated FECH alleles. In dominant cases (95%) the inheritance of a common hypomorphic IVS3-48C FECH allele trans to a deleterious FECH mutation reduces FECH activity below a critical threshold. The frequency of the IVS3-48C allele differs widely from the Japanese (45%), to Black West Africans (<1%) populations. These differences in the frequency of this single common SNP account for the prevalence of overt EPP in different countries and for the absence of EPP in Black Africans. The phylogenic origin of the IVS3-48C haplotypes strongly suggests that the IVS3-48C allele arose from a single recent mutational event that occurred 60 Kyears ago. Acquired somatic mutation of FECH secondary to myeloid disease may also exceptionally cause EPP (<1%). Finally, about 4% of unrelated EPP patients have X-linked dominant protoporphyria (XLDPP) (MIM 300752) caused by gain-of-function mutations in the ALAS2 gene leading to an increased erythroid heme biosynthesis and subsequently an accumulation of protoporphyrin without any FECH deficiency.
char = I;code = 73
char = n;code = 110
char = h;code = 104
char = e;code = 101
char = r;code = 114
char = i;code = 105
char = t;code = 116
char = a;code = 97
char = n;code = 110
char = c;code = 99
char = e;code = 101
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char = i;code = 105
char = n;code = 110
char = ;code = 32
char = e;code = 101
char = r;code = 114
char = y;code = 121
char = t;code = 116
char = h;code = 104
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char = o;code = 111
char = p;code = 112
char = o;code = 111
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char = e;code = 101
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char = i;code = 105
char = c;code = 99
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char = p;code = 112
char = r;code = 114
char = o;code = 111
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char = o;code = 111
char = p;code = 112
char = o;code = 111
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char = p;code = 112
char = h;code = 104
char = y;code = 121
char = r;code = 114
char = i;code = 105
char = a;code = 97
char = ];code = 93
char = .;code = 46
result sql = [Inheritance in erythropoietic protoporphyria].
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char = y;code = 121
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char = e;code = 101
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char = i;code = 105
char = s;code = 115
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char = h;code = 104
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char = u;code = 117
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char = c;code = 99
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char = m;code = 109
char = u;code = 117
char = l;code = 108
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char = t;code = 116
char = i;code = 105
char = o;code = 111
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char = o;code = 111
char = f;code = 102
char = ;code = 32
char = p;code = 112
char = r;code = 114
char = o;code = 111
char = t;code = 116
char = o;code = 111
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char = o;code = 111
char = r;code = 114
char = p;code = 112
char = h;code = 104
char = y;code = 121
char = r;code = 114
char = i;code = 105
char = n;code = 110
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char = I;code = 73
char = X;code = 88
char = ;code = 32
char = i;code = 105
char = n;code = 110
char = ;code = 32
char = e;code = 101
char = r;code = 114
char = y;code = 121
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char = h;code = 104
char = r;code = 114
char = o;code = 111
char = i;code = 105
char = d;code = 100
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char = c;code = 99
char = e;code = 101
char = l;code = 108
char = l;code = 108
char = s;code = 115
char = ,;code = 44
char = ;code = 32
char = p;code = 112
char = l;code = 108
char = a;code = 97
char = s;code = 115
char = m;code = 109
char = a;code = 97
char = ,;code = 44
char = ;code = 32
char = s;code = 115
char = k;code = 107
char = i;code = 105
char = n;code = 110
char = ;code = 32
char = a;code = 97
char = n;code = 110
char = d;code = 100
char = ;code = 32
char = l;code = 108
char = i;code = 105
char = v;code = 118
char = e;code = 101
char = r;code = 114
char = .;code = 46
char = ;code = 32
char = E;code = 69
char = P;code = 80
char = P;code = 80
char = ;code = 32
char = l;code = 108
char = e;code = 101
char = a;code = 97
char = d;code = 100
char = s;code = 115
char = ;code = 32
char = t;code = 116
char = o;code = 111
char = ;code = 32
char = a;code = 97
char = c;code = 99
char = u;code = 117
char = t;code = 116
char = e;code = 101
char = ;code = 32
char = p;code = 112
char = h;code = 104
char = o;code = 111
char = t;code = 116
char = o;code = 111
char = s;code = 115
char = e;code = 101
char = n;code = 110
char = s;code = 115
char = i;code = 105
char = t;code = 116
char = i;code = 105
char = v;code = 118
char = i;code = 105
char = t;code = 116
char = y;code = 121
char = ;code = 32
char = a;code = 97
char = n;code = 110
char = d;code = 100
char = ,;code = 44
char = ;code = 32
char = i;code = 105
char = n;code = 110
char = ;code = 32
char = a;code = 97
char = b;code = 98
char = o;code = 111
char = u;code = 117
char = t;code = 116
char = ;code = 32
char = 2;code = 50
char = %;code = 37
char = ;code = 32
char = o;code = 111
char = f;code = 102
char = ;code = 32
char = p;code = 112
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = e;code = 101
char = n;code = 110
char = t;code = 116
char = s;code = 115
char = ,;code = 44
char = ;code = 32
char = l;code = 108
char = i;code = 105
char = v;code = 118
char = e;code = 101
char = r;code = 114
char = ;code = 32
char = d;code = 100
char = i;code = 105
char = s;code = 115
char = e;code = 101
char = a;code = 97
char = s;code = 115
char = e;code = 101
char = .;code = 46
char = ;code = 32
char = E;code = 69
char = P;code = 80
char = P;code = 80
char = ;code = 32
char = i;code = 105
char = s;code = 115
char = ;code = 32
char = a;code = 97
char = ;code = 32
char = c;code = 99
char = o;code = 111
char = m;code = 109
char = p;code = 112
char = l;code = 108
char = e;code = 101
char = x;code = 120
char = ;code = 32
char = s;code = 115
char = y;code = 121
char = n;code = 110
char = d;code = 100
char = r;code = 114
char = o;code = 111
char = m;code = 109
char = e;code = 101
char = ;code = 32
char = i;code = 105
char = n;code = 110
char = ;code = 32
char = w;code = 119
char = h;code = 104
char = i;code = 105
char = c;code = 99
char = h;code = 104
char = ;code = 32
char = t;code = 116
char = w;code = 119
char = o;code = 111
char = ;code = 32
char = g;code = 103
char = e;code = 101
char = n;code = 110
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = a;code = 97
char = r;code = 114
char = e;code = 101
char = ;code = 32
char = i;code = 105
char = n;code = 110
char = d;code = 100
char = e;code = 101
char = p;code = 112
char = e;code = 101
char = n;code = 110
char = d;code = 100
char = e;code = 101
char = n;code = 110
char = t;code = 116
char = l;code = 108
char = y;code = 121
char = ;code = 32
char = i;code = 105
char = n;code = 110
char = v;code = 118
char = o;code = 111
char = l;code = 108
char = v;code = 118
char = e;code = 101
char = d;code = 100
char = :;code = 58
char = ;code = 32
char = F;code = 70
char = E;code = 69
char = C;code = 67
char = H;code = 72
char = ;code = 32
char = a;code = 97
char = n;code = 110
char = d;code = 100
char = ;code = 32
char = A;code = 65
char = L;code = 76
char = A;code = 65
char = S;code = 83
char = 2;code = 50
char = .;code = 46
char = ;code = 32
char = M;code = 77
char = o;code = 111
char = r;code = 114
char = e;code = 101
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = a;code = 97
char = n;code = 110
char = ;code = 32
char = 9;code = 57
char = 6;code = 54
char = %;code = 37
char = ;code = 32
char = o;code = 111
char = f;code = 102
char = ;code = 32
char = u;code = 117
char = n;code = 110
char = r;code = 114
char = e;code = 101
char = l;code = 108
char = a;code = 97
char = t;code = 116
char = e;code = 101
char = d;code = 100
char = ;code = 32
char = E;code = 69
char = P;code = 80
char = P;code = 80
char = ;code = 32
char = p;code = 112
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = e;code = 101
char = n;code = 110
char = t;code = 116
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 = f;code = 102
char = e;code = 101
char = r;code = 114
char = r;code = 114
char = o;code = 111
char = c;code = 99
char = h;code = 104
char = e;code = 101
char = l;code = 108
char = a;code = 97
char = t;code = 116
char = a;code = 97
char = s;code = 115
char = e;code = 101
char = ;code = 32
char = (;code = 40
char = F;code = 70
char = E;code = 69
char = C;code = 67
char = H;code = 72
char = );code = 41
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 = 32
char = (;code = 40
char = M;code = 77
char = I;code = 73
char = M;code = 77
char = ;code = 32
char = 1;code = 49
char = 7;code = 55
char = 7;code = 55
char = 0;code = 48
char = 0;code = 48
char = 0;code = 48
char = );code = 41
char = .;code = 46
char = ;code = 32
char = F;code = 70
char = o;code = 111
char = u;code = 117
char = r;code = 114
char = ;code = 32
char = p;code = 112
char = e;code = 101
char = r;code = 114
char = c;code = 99
char = e;code = 101
char = n;code = 110
char = t;code = 116
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 = m;code = 109
char = ;code = 32
char = p;code = 112
char = r;code = 114
char = e;code = 101
char = s;code = 115
char = e;code = 101
char = n;code = 110
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 = u;code = 117
char = t;code = 116
char = o;code = 111
char = s;code = 115
char = o;code = 111
char = m;code = 109
char = a;code = 97
char = l;code = 108
char = ;code = 32
char = r;code = 114
char = e;code = 101
char = c;code = 99
char = e;code = 101
char = s;code = 115
char = s;code = 115
char = i;code = 105
char = v;code = 118
char = e;code = 101
char = ;code = 32
char = i;code = 105
char = n;code = 110
char = h;code = 104
char = e;code = 101
char = r;code = 114
char = i;code = 105
char = t;code = 116
char = a;code = 97
char = n;code = 110
char = c;code = 99
char = e;code = 101
char = ;code = 32
char = w;code = 119
char = i;code = 105
char = t;code = 116
char = h;code = 104
char = ;code = 32
char = t;code = 116
char = w;code = 119
char = o;code = 111
char = ;code = 32
char = m;code = 109
char = u;code = 117
char = t;code = 116
char = a;code = 97
char = t;code = 116
char = e;code = 101
char = d;code = 100
char = ;code = 32
char = F;code = 70
char = E;code = 69
char = C;code = 67
char = H;code = 72
char = ;code = 32
char = a;code = 97
char = l;code = 108
char = l;code = 108
char = e;code = 101
char = l;code = 108
char = e;code = 101
char = s;code = 115
char = .;code = 46
char = ;code = 32
char = I;code = 73
char = n;code = 110
char = ;code = 32
char = d;code = 100
char = o;code = 111
char = m;code = 109
char = i;code = 105
char = n;code = 110
char = a;code = 97
char = n;code = 110
char = t;code = 116
char = ;code = 32
char = c;code = 99
char = a;code = 97
char = s;code = 115
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = (;code = 40
char = 9;code = 57
char = 5;code = 53
char = %;code = 37
char = );code = 41
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = e;code = 101
char = ;code = 32
char = i;code = 105
char = n;code = 110
char = h;code = 104
char = e;code = 101
char = r;code = 114
char = i;code = 105
char = t;code = 116
char = a;code = 97
char = n;code = 110
char = c;code = 99
char = e;code = 101
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char = o;code = 111
char = f;code = 102
char = ;code = 32
char = a;code = 97
char = ;code = 32
char = c;code = 99
char = o;code = 111
char = m;code = 109
char = m;code = 109
char = o;code = 111
char = n;code = 110
char = ;code = 32
char = h;code = 104
char = y;code = 121
char = p;code = 112
char = o;code = 111
char = m;code = 109
char = o;code = 111
char = r;code = 114
char = p;code = 112
char = h;code = 104
char = i;code = 105
char = c;code = 99
char = ;code = 32
char = I;code = 73
char = V;code = 86
char = S;code = 83
char = 3;code = 51
char = -;code = 45
char = 4;code = 52
char = 8;code = 56
char = C;code = 67
char = ;code = 32
char = F;code = 70
char = E;code = 69
char = C;code = 67
char = H;code = 72
char = ;code = 32
char = a;code = 97
char = l;code = 108
char = l;code = 108
char = e;code = 101
char = l;code = 108
char = e;code = 101
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char = t;code = 116
char = r;code = 114
char = a;code = 97
char = n;code = 110
char = s;code = 115
char = ;code = 32
char = t;code = 116
char = o;code = 111
char = ;code = 32
char = a;code = 97
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char = d;code = 100
char = e;code = 101
char = l;code = 108
char = e;code = 101
char = t;code = 116
char = e;code = 101
char = r;code = 114
char = i;code = 105
char = o;code = 111
char = u;code = 117
char = s;code = 115
char = ;code = 32
char = F;code = 70
char = E;code = 69
char = C;code = 67
char = H;code = 72
char = ;code = 32
char = m;code = 109
char = u;code = 117
char = t;code = 116
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = o;code = 111
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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 = s;code = 115
char = ;code = 32
char = F;code = 70
char = E;code = 69
char = C;code = 67
char = H;code = 72
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
char = ;code = 32
char = b;code = 98
char = e;code = 101
char = l;code = 108
char = o;code = 111
char = w;code = 119
char = ;code = 32
char = a;code = 97
char = ;code = 32
char = c;code = 99
char = r;code = 114
char = i;code = 105
char = t;code = 116
char = i;code = 105
char = c;code = 99
char = a;code = 97
char = l;code = 108
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = r;code = 114
char = e;code = 101
char = s;code = 115
char = h;code = 104
char = o;code = 111
char = l;code = 108
char = d;code = 100
char = .;code = 46
char = ;code = 32
char = T;code = 84
char = h;code = 104
char = e;code = 101
char = ;code = 32
char = f;code = 102
char = r;code = 114
char = e;code = 101
char = q;code = 113
char = u;code = 117
char = e;code = 101
char = n;code = 110
char = c;code = 99
char = y;code = 121
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 = ;code = 32
char = I;code = 73
char = V;code = 86
char = S;code = 83
char = 3;code = 51
char = -;code = 45
char = 4;code = 52
char = 8;code = 56
char = C;code = 67
char = ;code = 32
char = a;code = 97
char = l;code = 108
char = l;code = 108
char = e;code = 101
char = l;code = 108
char = e;code = 101
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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 = s;code = 115
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char = w;code = 119
char = i;code = 105
char = d;code = 100
char = e;code = 101
char = l;code = 108
char = y;code = 121
char = ;code = 32
char = f;code = 102
char = r;code = 114
char = o;code = 111
char = m;code = 109
char = ;code = 32
char = t;code = 116
char = h;code = 104
char = e;code = 101
char = ;code = 32
char = J;code = 74
char = a;code = 97
char = p;code = 112
char = a;code = 97
char = n;code = 110
char = e;code = 101
char = s;code = 115
char = e;code = 101
char = ;code = 32
char = (;code = 40
char = 4;code = 52
char = 5;code = 53
char = %;code = 37
char = );code = 41
char = ,;code = 44
char = ;code = 32
char = t;code = 116
char = o;code = 111
char = ;code = 32
char = B;code = 66
char = l;code = 108
char = a;code = 97
char = c;code = 99
char = k;code = 107
char = ;code = 32
char = W;code = 87
char = e;code = 101
char = s;code = 115
char = t;code = 116
char = ;code = 32
char = A;code = 65
char = f;code = 102
char = r;code = 114
char = i;code = 105
char = c;code = 99
char = a;code = 97
char = n;code = 110
char = s;code = 115
char = ;code = 32
char = (;code = 40
char = <;code = 60
char = 1;code = 49
char = %;code = 37
char = );code = 41
char = ;code = 32
char = p;code = 112
char = o;code = 111
char = p;code = 112
char = u;code = 117
char = l;code = 108
char = a;code = 97
char = t;code = 116
char = i;code = 105
char = o;code = 111
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char = s;code = 115
char = .;code = 46
char = ;code = 32
char = T;code = 84
char = h;code = 104
char = e;code = 101
char = s;code = 115
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char = d;code = 100
char = i;code = 105
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char = f;code = 102
char = e;code = 101
char = r;code = 114
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char = e;code = 101
char = s;code = 115
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char = i;code = 105
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char = t;code = 116
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char = e;code = 101
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char = f;code = 102
char = r;code = 114
char = e;code = 101
char = q;code = 113
char = u;code = 117
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 = t;code = 116
char = h;code = 104
char = i;code = 105
char = s;code = 115
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char = s;code = 115
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char = n;code = 110
char = g;code = 103
char = l;code = 108
char = e;code = 101
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char = c;code = 99
char = o;code = 111
char = m;code = 109
char = m;code = 109
char = o;code = 111
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char = S;code = 83
char = N;code = 78
char = P;code = 80
char = ;code = 32
char = a;code = 97
char = c;code = 99
char = c;code = 99
char = o;code = 111
char = u;code = 117
char = n;code = 110
char = t;code = 116
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char = f;code = 102
char = o;code = 111
char = r;code = 114
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char = t;code = 116
char = h;code = 104
char = e;code = 101
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char = p;code = 112
char = r;code = 114
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char = v;code = 118
char = a;code = 97
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char = e;code = 101
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char = c;code = 99
char = e;code = 101
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char = o;code = 111
char = f;code = 102
char = ;code = 32
char = o;code = 111
char = v;code = 118
char = e;code = 101
char = r;code = 114
char = t;code = 116
char = ;code = 32
char = E;code = 69
char = P;code = 80
char = P;code = 80
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char = i;code = 105
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char = d;code = 100
char = i;code = 105
char = f;code = 102
char = f;code = 102
char = e;code = 101
char = r;code = 114
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char = n;code = 110
char = t;code = 116
char = ;code = 32
char = c;code = 99
char = o;code = 111
char = u;code = 117
char = n;code = 110
char = t;code = 116
char = r;code = 114
char = i;code = 105
char = e;code = 101
char = s;code = 115
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char = a;code = 97
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char = d;code = 100
char = ;code = 32
char = f;code = 102
char = o;code = 111
char = r;code = 114
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char = t;code = 116
char = h;code = 104
char = e;code = 101
char = ;code = 32
char = a;code = 97
char = b;code = 98
char = s;code = 115
char = e;code = 101
char = n;code = 110
char = c;code = 99
char = e;code = 101
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char = o;code = 111
char = f;code = 102
char = ;code = 32
char = E;code = 69
char = P;code = 80
char = P;code = 80
char = ;code = 32
char = i;code = 105
char = n;code = 110
char = ;code = 32
char = B;code = 66
char = l;code = 108
char = a;code = 97
char = c;code = 99
char = k;code = 107
char = ;code = 32
char = A;code = 65
char = f;code = 102
char = r;code = 114
char = i;code = 105
char = c;code = 99
char = a;code = 97
char = n;code = 110
char = s;code = 115
char = .;code = 46
char = ;code = 32
char = T;code = 84
char = h;code = 104
char = e;code = 101
char = ;code = 32
char = p;code = 112
char = h;code = 104
char = y;code = 121
char = l;code = 108
char = o;code = 111
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char = e;code = 101
char = n;code = 110
char = i;code = 105
char = c;code = 99
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char = o;code = 111
char = r;code = 114
char = i;code = 105
char = g;code = 103
char = i;code = 105
char = n;code = 110
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 = ;code = 32
char = I;code = 73
char = V;code = 86
char = S;code = 83
char = 3;code = 51
char = -;code = 45
char = 4;code = 52
char = 8;code = 56
char = C;code = 67
char = ;code = 32
char = h;code = 104
char = a;code = 97
char = p;code = 112
char = l;code = 108
char = o;code = 111
char = t;code = 116
char = y;code = 121
char = p;code = 112
char = e;code = 101
char = s;code = 115
char = ;code = 32
char = s;code = 115
char = t;code = 116
char = r;code = 114
char = o;code = 111
char = n;code = 110
char = g;code = 103
char = l;code = 108
char = y;code = 121
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 = s;code = 115
char = ;code = 32
char = t;code = 116
char = h;code = 104
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result sql = Erythropoietic protoporphyria (EPP) is an inherited disorder of heme biosynthesis that results from an accumulation of protoporphyrin IX in erythroid cells, plasma, skin and liver. EPP leads to acute photosensitivity and, in about 2% of patients, liver disease. EPP is a complex syndrome in which two genes are independently involved: FECH and ALAS2. More than 96% of unrelated EPP patients have ferrochelatase (FECH) deficiency (MIM 177000). Four percent of them present with autosomal recessive inheritance with two mutated FECH alleles. In dominant cases (95%) the inheritance of a common hypomorphic IVS3-48C FECH allele trans to a deleterious FECH mutation reduces FECH activity below a critical threshold. The frequency of the IVS3-48C allele differs widely from the Japanese (45%), to Black West Africans (<1%) populations. These differences in the frequency of this single common SNP account for the prevalence of overt EPP in different countries and for the absence of EPP in Black Africans. The phylogenic origin of the IVS3-48C haplotypes strongly suggests that the IVS3-48C allele arose from a single recent mutational event that occurred 60 Kyears ago. Acquired somatic mutation of FECH secondary to myeloid disease may also exceptionally cause EPP (<1%). Finally, about 4% of unrelated EPP patients have X-linked dominant protoporphyria (XLDPP) (MIM 300752) caused by gain-of-function mutations in the ALAS2 gene leading to an increased erythroid heme biosynthesis and subsequently an accumulation of protoporphyrin without any FECH deficiency.
Publication: Pathol. Biol. 2010 Oct ;58(5)::372-80. :doi: 10.1016/j.patbio.2010.01.007.
Publication Date: 2010 Oct
Study Author(s): Schmitt, C;Ducamp, S;Gouya, L;Deybach, J-C;Puy, H;
Institution: Centre Français des Porphyries, Service de Biochimie, Hôpital Louis-Mourier, AP-HP, 178 rue des Renouillers, 92701 Colombes cedex, France.
Shortcut link to this study:
Publication Date: 2010 Oct
Study Author(s): Schmitt, C;Ducamp, S;Gouya, L;Deybach, J-C;Puy, H;
Institution: Centre Français des Porphyries, Service de Biochimie, Hôpital Louis-Mourier, AP-HP, 178 rue des Renouillers, 92701 Colombes cedex, France.
Shortcut link to this study:
Erythropoietic protoporphyria (EPP) is an inherited disorder of heme biosynthesis that results from an accumulation of protoporphyrin IX in erythroid cells, plasma, skin and liver. EPP leads to acute photosensitivity and, in about 2% of patients, liver disease. EPP is a complex syndrome in which two genes are independently involved: FECH and ALAS2. More than 96% of unrelated EPP patients have ferrochelatase (FECH) deficiency (MIM 177000). Four percent of them present with autosomal recessive inheritance with two mutated FECH alleles. In dominant cases (95%) the inheritance of a common hypomorphic IVS3-48C FECH allele trans to a deleterious FECH mutation reduces FECH activity below a critical threshold. The frequency of the IVS3-48C allele differs widely from the Japanese (45%), to Black West Africans (<1%) populations. These differences in the frequency of this single common SNP account for the prevalence of overt EPP in different countries and for the absence of EPP in Black Africans. The phylogenic origin of the IVS3-48C haplotypes strongly suggests that the IVS3-48C allele arose from a single recent mutational event that occurred 60 Kyears ago. Acquired somatic mutation of FECH secondary to myeloid disease may also exceptionally cause EPP (<1%). Finally, about 4% of unrelated EPP patients have X-linked dominant protoporphyria (XLDPP) (MIM 300752) caused by gain-of-function mutations in the ALAS2 gene leading to an increased erythroid heme biosynthesis and subsequently an accumulation of protoporphyrin without any FECH deficiency.
PMID: 20850938
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