SAB2108553
Anti-FAH
IgG fraction of antiserum
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About This Item
Código UNSPSC:
12352203
NACRES:
NA.41
Produtos recomendados
fonte biológica
rabbit
Nível de qualidade
conjugado
unconjugated
forma do anticorpo
IgG fraction of antiserum
tipo de produto de anticorpo
primary antibodies
clone
polyclonal
forma
buffered aqueous solution
peso molecular
46 kDa
reatividade de espécies
dog, horse, human, mouse, rat
concentração
0.5-1 mg/mL
técnica(s)
immunoblotting: suitable
immunohistochemistry: suitable
nº de adesão
NM_000137
nº de adesão UniProt
Condições de expedição
wet ice
temperatura de armazenamento
−20°C
modificação pós-traducional do alvo
unmodified
Informações sobre genes
human ... FAH(2184)
Descrição geral
The FAH gene is mapped to human chromosome 15q25.1. The encoded protein consists of 419 amino acids and 14 coding exons.
Imunogênio
Synthetic peptide directed towards the C terminal region of human FAH
Ações bioquímicas/fisiológicas
FAH (fumarylacetoacetate hydrolase) catalyzes the last step in tyrosine catabolic pathway. FAH deficiency leads to hereditary tyrosinemia type 1. FAH mutations eventually lead to Renal Fanconi Syndrome, due to severe liver cirrhosis and renal tubular acidosis caused by accumulation of toxic metabolites such as fumarylacetoacetate.
FAH is the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia.This gene encodes the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT).
Sequência
Synthetic peptide located within the following region: AATICKSNFKYMYWTMLQQLTHHSVNGCNLRPGDLLASGTISGPEPENFG
forma física
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Exoneração de responsabilidade
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Código de classe de armazenamento
10 - Combustible liquids
Classe de risco de água (WGK)
WGK 3
Ponto de fulgor (°F)
Not applicable
Ponto de fulgor (°C)
Not applicable
Certificados de análise (COA)
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Já possui este produto?
Encontre a documentação dos produtos que você adquiriu recentemente na biblioteca de documentos.
Molecular Aspects of the FAH Mutations Involved in HT1 Disease.
Morrow G, et al.
Advances in Experimental Medicine and Biology, 25-48 (2017)
Silent tyrosinemia type I without elevated tyrosine or succinylacetone associated with liver cirrhosis and hepatocellular carcinoma.
Blackburn P R, et al.
Human Mutation, 37(10), 1097-1105 (2016)
Marco De Giorgi et al.
Molecular therapy. Methods & clinical development, 21, 656-669 (2021-06-19)
Clinical application of somatic genome editing requires therapeutics that are generalizable to a broad range of patients. Targeted insertion of promoterless transgenes can ensure that edits are permanent and broadly applicable while minimizing risks of off-target integration. In the liver
Biochemical and molecular diagnosis of tyrosinemia type I with two novel FAH mutations in a Hong Kong chinese patient: Recommendation for expanded newborn screening in Hong Kong
Mak CM, et al.
Clinical Biochemistry, 46(1-2), 155-159 (2013)
Mercedes Barzi et al.
Nature communications, 15(1), 1955-1955 (2024-03-05)
Clinical translation of AAV-mediated gene therapy requires preclinical development across different experimental models, often confounded by variable transduction efficiency. Here, we describe a human liver chimeric transgene-free Il2rg-/-/Rag2-/-/Fah-/-/Aavr-/- (TIRFA) mouse model overcoming this translational roadblock, by combining liver humanization with
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