biological source
rabbit
conjugate
unconjugated
antibody form
IgG fraction of antiserum
antibody product type
primary antibodies
clone
polyclonal
form
buffered aqueous solution
mol wt
antigen ~46 kDa
species reactivity
human
technique(s)
western blot: 1:500-1:2,000
UniProt accession no.
shipped in
dry ice
storage temp.
−20°C
Gene Information
human ... FAH(2184)
Immunogen
synthetic peptide corresponding to amino acids 366-380 of human FAH
Application
Anti-FAH (366-380) antibody is suitable for western blot at a dilution of 1:500-1:2,000.
Yale Center for High Throughput Cell Biology IF-tested antibodies. Each antibody is tested by immunofluorescence against HUVEC cells using the Yale HTCB IF protocol. To learn more about us and Yale Center for High Throughput Cell Biology partnership, visit sigma.com/htcb-if.
Yale Center for High Throughput Cell Biology IF-tested antibodies. Each antibody is tested by immunofluorescence against HUVEC cells using the Yale HTCB IF protocol. To learn more about us and Yale Center for High Throughput Cell Biology partnership, visit sigma.com/htcb-if.
Biochem/physiol Actions
FAH is the terminal enzyme in the tyrosine catabolism pathway. It is mainly expressed in the liver and kidney. Deficiency in FAH results Type 1 hereditary tyrosinemia (HT), an autosomal recessive disorder.
Physical form
Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide
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存储类别
12 - Non Combustible Liquids
wgk
WGK 2
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
法规信息
新产品
此项目有
Y Labelle et al.
Biochimica et biophysica acta, 1180(3), 250-256 (1993-01-22)
Fumarylacetoacetate hydrolase (FAH) is the terminal enzyme in the catabolic pathway of tyrosine. This enzyme which is mainly expressed in the liver and kidney is deficient in hereditary tyrosinemia type 1. As some affected individuals present neurologic abnormalities, we studied
Y Labelle et al.
Human molecular genetics, 2(7), 941-946 (1993-07-01)
Hereditary tyrosinemia type 1 is an autosomal recessive disease caused by a deficiency of the last enzyme in the catabolic pathway of tyrosine, fumarylacetoacetate hydrolase (FAH). To analyze the mutations involved in this disease, and as a first step towards
Faiqa Imtiaz et al.
Molecular genetics and metabolism, 104(4), 688-690 (2011-07-19)
Hereditary Tyrosinemia Type 1 (HT1) is an autosomal recessive disorder resulting from a deficiency of fumarylacetoacetase caused by mutations in the fumarylacetoacetate hydrolase (FAH) gene. We detected 11 novel and 6 previously described pathogenic mutations in a cohort of 43
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