Gene Score gda Association Type Type Original DB Sentence supporting the association PMID PMID Year
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Mutational hotspot in the human biotinidase gene causes profound biotinidase deficiency. 7550325 1995
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 GeneticVariation disease CLINVAR Deletion/insertion mutation that causes biotinidase deficiency may result from the formation of a quasipalindromic structure. 8894703 1996
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Arg538 to Cys mutation in a CpG dinucleotide of the human biotinidase gene is the second most common cause of profound biotinidase deficiency in symptomatic children. 9099842 1997
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Profound biotinidase deficiency caused by a point mutation that creates a downstream cryptic 3' splice acceptor site within an exon of the human biotinidase gene. 9158148 1997
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Mutation (Q456H) is the most common cause of profound biotinidase deficiency in children ascertained by newborn screening in the United States. 9232193 1997
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Profound biotinidase deficiency in two asymptomatic adults. 9375914 1997
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Mutations in the human biotinidase gene that cause profound biotinidase deficiency in symptomatic children: molecular, biochemical, and clinical analysis. 9396567 1997
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 GeneticVariation disease CLINVAR Mutations in the human biotinidase gene that cause profound biotinidase deficiency in symptomatic children: molecular, biochemical, and clinical analysis. 9396567 1997
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Delayed-onset profound biotinidase deficiency. 9506660 1998
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Partial biotinidase deficiency is usually due to the D444H mutation in the biotinidase gene. 9654207 1998
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Double mutation (A171T and D444H) is a common cause of profound biotinidase deficiency in children ascertained by newborn screening the the United States. Mutations in brief no. 128. Online. 10206677 1998
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR [Biotinidase deficiency: importance of its neonatal diagnosis and early treatment]. 10394193 1999
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 GeneticVariation disease CLINVAR Mutations causing profound biotinidase deficiency in children ascertained by newborn screening in the United States occur at different frequencies than in symptomatic children. 10400129 1999
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Mutations causing profound biotinidase deficiency in children ascertained by newborn screening in the United States occur at different frequencies than in symptomatic children. 10400129 1999
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Novel mutations cause biotinidase deficiency in Turkish children. 10801053 2000
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 GeneticVariation disease CLINVAR Novel mutations cause biotinidase deficiency in Turkish children. 10801053 2000
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Novel mutations in children with profound biotinidase deficiency from Saudi Arabia. 10801060 2000
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Molecular characterisation of 34 patients with biotinidase deficiency ascertained by newborn screening and family investigation. 11313766 2001
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 GeneticVariation disease CLINVAR The nitrilase superfamily: classification, structure and function. 11380987 2001
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 GeneticVariation disease CLINVAR Thus far 61 mutations in three of the four exons of the BTD and one mutation in an intron gene that cause profound BTD deficiency have been reported. 11668630 2001
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Hearing loss is a common feature of symptomatic children with profound biotinidase deficiency. 11865279 2002
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Two new mutations in children affected by partial biotinidase deficiency ascertained by newborn screening. 12227467 2002
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Seventeen novel mutations that cause profound biotinidase deficiency. 12359137 2003
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 GeneticVariation disease CLINVAR Seventeen novel mutations that cause profound biotinidase deficiency. 12359137 2003
Entrez Id: 686
Gene Symbol: BTD
BTD
1.000 CausalMutation disease CLINVAR Real time PCR assays to detect common mutations in the biotinidase gene and application of mutational analysis to newborn screening for biotinidase deficiency. 12618081 2003