Abcam, ab252955, Anti-UROS antibody

Size: 100µL
Rabbit Polyclonal UROS antibody. Suitable for IHC-P, WB, ICC/IF and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human UROS aa 100-250.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:ICC/IF, IHC-P, WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human UROS aa 100-250. The exact immunogen used to generate this antibody is proprietary information.P10746

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Immunogen, Storage buffer-pH: 7.2Preservative: 0.02% Sodium azideConstituents: PBS, 40% Glycerol (glycerin, glycerine), 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.
Uroporphyrinogen III synthase commonly known as UROS is an enzyme involved in heme biosynthesis. It catalyzes the conversion of hydroxymethylbilane to uroporphyrinogen III facilitating a ring closure and isomerization. UROS has a molecular mass of approximately 29 kDa. It is predominantly expressed in the liver bone marrow and erythroid tissues where heme production is essential. The enzyme is vital for maintaining proper levels of porphyrins in cells and ensuring the biosynthesis of heme an important component of hemoglobin.
Biological function summary
Uroporphyrinogen III synthase plays a role in heme production converting linear tetrapyrroles into the cyclic form needed for biosynthesis. This enzyme does not function as part of a larger complex but rather works independently to catalyze its specific reaction in porphyrin metabolism. Proper function of UROS is necessary to sustain the production of heme which is critical not only for oxygen transport in the blood but also for other heme-dependent processes in various tissues.
Pathways
UROS is an essential component of the heme biosynthesis pathway. In this pathway it acts between the steps that involve hydroxymethylbilane synthase (HMBS) and uroporphyrinogen III decarboxylase (UROD). This metabolic pathway is important for the production of heme which is a core component of hemoglobin. HMBS synthesizes the precursor transformed by UROS while UROD further modifies the product of UROS to continue the biosynthesis process emphasizing the enzyme's importance in maintaining the heme biosynthesis pathway's integrity.
Mutations or deficiencies in UROS are linked to a rare inherited condition called congenital erythropoietic porphyria (CEP) also known as Günther's disease. This disorder results from the accumulation of porphyrins owing to impaired heme biosynthesis. Abnormal function in UROS leads to a buildup of its substrates causing severe skin photosensitivity and hemolytic anemia among other symptoms. The functional relationship between UROS and HMBS or UROD in the heme biosynthesis pathway illustrates the enzyme's significant role in preventing the development of such heme-related disorders.