Rabbit FAH Polyclonal Antibody | anti-FAH antibody

FAH (Fumarylacetoacetate Hydrolase) (FITC)

Cat. Num. AAA6416764

• Reactivity: Human, Mouse, Rat
• Applications: Western Blot
• Purity: Purified by affinity chromatography.
• Synonyms: FAH; Polyclonal Antibody; FAH (Fumarylacetoacetate Hydrolase) (FITC); anti-FAH antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes FAH. Species Crossreactivity: human, mouse, rat
• Purity/Purification: Purified by affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).
• Concentration: ~1mg/ml (varies by lot)

• Applicable Applications for anti-FAH antibody: Western Blot (WB)
• Application Notes: WB: 1:500-1:1000; Applications are based on unconjugated antibody.
• Immunogen: Recombinant protein corresponding to human FAH.
• Conjugate: FITC
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western blot analysis of extracts of mouse live cell line, using FAH antibody.)

Product Categories/Family for anti-FAH antibody

Antibodies; Enzymes

NCBI and Uniprot Product Information

• NCBI GI #: 119778
• NCBI GeneID: 2184
• UniProt Accession #: P16930; Q53XA7; B2R9X1; D3DW95
• Molecular Weight: 419
• NCBI Official Full Name: Fumarylacetoacetase
• NCBI Official Synonym Full Names: fumarylacetoacetate hydrolase (fumarylacetoacetase)
• NCBI Official Symbol: FAH
• NCBI Protein Information: fumarylacetoacetase; FAA; beta-diketonase
• UniProt Protein Name: Fumarylacetoacetase
• Protein Family: Fumarylacetoacetase
• UniProt Gene Name: FAH
• UniProt Synonym Gene Names: FAA
• UniProt Entry Name: FAAA_HUMAN

NCBI Description

This gene encodes the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT). [provided by RefSeq, Jul 2008]

Uniprot Description

FAH: Defects in FAH are the cause of tyrosinemia type 1 (TYRO1). An inborn error of metabolism characterized by elevations of tyrosine in the blood and urine, and hepatorenal manifestations. Typical features include hepatic necrosis, renal tubular injury, episodic weakness, self-mutilation, and seizures. Renal tubular dysfunction is associated with phosphate loss and hypophosphataemic rickets. Progressive liver disease can lead to the development of hepatocellular carcinoma. Dietary treatment with restriction of tyrosine and phenylalanine alleviates the rickets, but liver transplantation has so far been the only definite treatment. Belongs to the FAH family.

Protein type: Amino Acid Metabolism - tyrosine; EC 3.7.1.2; Hydrolase

Chromosomal Location of Human Ortholog: 15q25.1

Cellular Component: cytosol

Molecular Function: fumarylacetoacetase activity; metal ion binding

Biological Process: L-phenylalanine catabolic process; tyrosine catabolic process; arginine catabolic process

Disease: Tyrosinemia, Type I