SAB2108553

Anti-FAH

IgG fraction of antiserum

• Código UNSPSC: 12352203
• NACRES: NA.41

Propriedades

• fonte biológica: rabbit
• Nível de qualidade: 100
• 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: P16930
• Condições de expedição: wet ice
• temperatura de armazenamento: −20°C
• modificação pós-traducional do alvo: unmodified
• Informações sobre genes: human ... FAH(2184)

Descrição

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.

Informações de segurança

• 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