Abcam, ab317120, Anti-FAH antibody [HL1970]

Size: 100µL
Rabbit Monoclonal Fumarylacetoacetate hydrolase/FAA antibody. Suitable for WB, IHC-P and reacts with Transfected cell lysate, Human, Mouse, Rat samples. Immunogen corresponding to Recombinant Fragment Protein within Human FAH.
Key facts
Host species:Rabbit,
Clonality:Monoclonal,
Clone number:HL1970,
Isotype:IgG,
Carrier free:No,
Reacts with:Human, Mouse, Rat,
Applications:WB, IHC-PSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human FAH.P16930

Properties and Storage Information:
Form-Liquid, Storage buffer-pH: 7.4Constituents: PBS, Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
Fumarylacetoacetate hydrolase (FAH) sometimes known as FAA hydrolase is an enzyme involved in the last step of tyrosine degradation. It catalyzes the hydrolysis of fumarylacetoacetate into fumarate and acetoacetate facilitating the breakdown of amino acids. This enzyme typically has a molecular mass of around 46 kDa. FAH is mostly expressed in the liver and kidneys reflecting its role in detoxifying metabolic byproducts in these organs.
Biological function summary
FAH plays a critical role in the catabolism of the amino acid tyrosine. It functions as a monomer rather than part of a larger protein complex. The enzyme's activity ensures the conversion of potentially toxic intermediates into less harmful components that the body can either utilize or excrete. This conversion is essential for maintaining metabolic equilibrium and preventing the accumulation of toxic metabolites which can damage cells.
Pathways
FAH is integral to the tyrosine degradation pathway. The breakdown process involves several enzymes with FAH being the final step converting fumarylacetoacetate. Enzymes like homogentisate 12-dioxygenase which acts earlier in the pathway are related to FAH within this context. This pathway is important for energy production and the synthesis of important biomolecules making each step's function interconnected with cellular health and metabolism.
Defects in FAH activity lead to hereditary tyrosinemia type I a metabolic disorder characterized by the accumulation of toxic intermediates due to disrupted tyrosine degradation. This disorder results in severe liver and kidney damage if not managed appropriately. Other proteins such as tyrosine aminotransferase can also be affected by analogous disruptions in the same metabolic pathway contributing to metabolic diseases when their function becomes impaired through the pathway's imbalances.