Anti-FAH
SIGMA/SAB2108553 - IgG fraction of antiserum
Product Type: Product-on-demand
Immunohistochemistry Anti-FAH: Cat. No. SAB2108553: Immunohistochemistry of FAH in Human Kidney tissue with FAH antibody at 4-8 μg/mL.
Immunohistochemistry Immunohistochemistry of FAH in human liver, mouse KO with FAH antibody at 4-8 μg/mL
Immunoblotting Cell Type: Jurkat (Cat. No. SAB2108553). Lanes 1. Antibody dilution (concentration) 2.5 μg/mL in cell type Jurkat
Western Blotting Western Blot of FAH in Mouse Testis with FAH antibody at 1 μg/mL
| accession no. | NM_000137 |
| antibody form | IgG fraction of antiserum |
| antibody product type | primary antibodies |
| biological source | rabbit |
| clone | polyclonal |
| concentration | 0.5-1 mg/mL |
| conjugate | unconjugated |
| form | buffered aqueous solution |
| mol wt | 46 kDa |
| Quality Level | 100  |
| shipped in | wet ice |
| species reactivity | dog, horse, human, mouse, rat |
| storage temp. | −20°C |
| target post-translational modification | unmodified |
| technique(s) | immunoblotting: suitable |
| immunohistochemistry: suitable | |
| UniProt accession no. | P16930  |
| Biochem/physiol Actions: | 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. |
| Biochem/physiol Actions: | 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). |
| Disclaimer: | 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. |
| General description: | 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 |
| Other Notes: | Synthetic peptide located within the following region: AATICKSNFKYMYWTMLQQLTHHSV |
| Physical form: | Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose. |
| RIDADR | NONH for all modes of transport |
| WGK Germany | WGK 3 |
| Flash Point(F) | Not applicable |
| Flash Point(C) | Not applicable |
| Storage Temp. | −20°C |
| UNSPSC | 12352203 |