Anti-FAA/FAH Antibody Picoband®

SKU: A02072-1

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,IHC,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat
Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml, Human, Mouse, Rat
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: FAH

Specificity:

Background: Fumarylacetoacetase is an enzyme that in humans is encoded by the FAH gene located on chromosome 15. The FAH gene is thought to be involved in the catabolism of the amino acid phenylalanine in humans. This gene encodes the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT).

Immunogen: E.coli-derived human FAA/FAH recombinant protein (Position: M1-P342).

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Berube, D., Phaneuf, D., Tanguay, R. M., Gagne, R. Assignment of the fumarylacetoacetate hydrolase gene to chromosome 15q23-15q25. (Abstract) Cytogenet. Cell Genet. 51: 962 only, 1989. 2. Demers, S. I., Phaneuf, D., Tanguay, R. M. Hereditary tyrosinemia type I: strong association with haplotype 6 in French Canadians permits simple carrier detection and prenatal diagnosis. Am. J. Hum. Genet. 55: 327-333, 1994. 3. Grompe, M., Al-Dhalimy, M. Mutations of the fumarylacetoacetate hydrolase gene in four patients with tyrosinemia, type I. Hum. Mutat. 2: 85-93, 1993.

Uniprot ID: P16930

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 41 kDa

Calculated Molecular Weight:

Gene ID: 2184

Protein Name: Fumarylacetoacetase

Gene Full Name: fumarylacetoacetate hydrolase

Synonyms: Beta diketonase; FAA; FAH; Fumarylacetoacetase; Fumarylacetoacetate hydrolase