SAB2108553

Anti-FAH

IgG fraction of antiserum

• UNSPSC-Code: 12352203
• NACRES: NA.41

Eigenschaften

• Biologische Quelle: rabbit
• Qualitätsniveau: 100
• Konjugat: unconjugated
• Antikörperform: IgG fraction of antiserum
• Antikörper-Produkttyp: primary antibodies
• Klon: polyclonal
• Form: buffered aqueous solution
• Mol-Gew.: 46 kDa
• Speziesreaktivität: dog, horse, human, mouse, rat
• Konzentration: 0.5-1 mg/mL
• Methode(n): immunoblotting: suitable; immunohistochemistry: suitable
• Hinterlegungsnummer: NM_000137
• UniProt-Hinterlegungsnummer: P16930
• Versandbedingung: wet ice
• Lagertemp.: −20°C
• Posttranslationale Modifikation Target: unmodified
• Angaben zum Gen: human ... FAH(2184)

Beschreibung

Allgemeine Beschreibung

The FAH gene is mapped to human chromosome 15q25.1. The encoded protein consists of 419 amino acids and 14 coding exons.

Immunogen

Synthetic peptide directed towards the C terminal region of human FAH

Biochem./physiol. Wirkung

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).

Sequenz

Synthetic peptide located within the following region: AATICKSNFKYMYWTMLQQLTHHSVNGCNLRPGDLLASGTISGPEPENFG

Physikalische Form

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

Haftungsausschluss

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.

Sicherheitshinweise

• Lagerklassenschlüssel: 10 - Combustible liquids
• WGK: WGK 3
• Flammpunkt (°F): Not applicable
• Flammpunkt (°C): Not applicable