Cell Biology Antibodies 18

Anti-UROS Antibody (CAB18390)

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SKU:
CAB18390
Product Type:
Antibody
Reactivity:
Human
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology

Description

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Antibody Name:Anti-UROS Antibody
Antibody SKU:CAB18390
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human
Host Species:Rabbit
Immunogen:Recombinant protein of human UROS.
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human
Positive Samples:
Immunogen:Recombinant protein of human UROS.
Purification Method:Affinity purification
Storage Buffer:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:7390
Uniprot:P10746
Cellular Location:
Calculated MW:
Observed MW:Refer to figures
Synonyms:UROIIIS, UROS
Background:
UniProt Protein Function:UROS: Catalyzes cyclization of the linear tetrapyrrole, hydroxymethylbilane, to the macrocyclic uroporphyrinogen III, the branch point for the various sub-pathways leading to the wide diversity of porphyrins. Porphyrins act as cofactors for a multitude of enzymes that perform a variety of processes within the cell such as methionine synthesis (vitamin B12) or oxygen transport (heme). Defects in UROS are the cause of congenital erythropoietic porphyria (CEP); also known as Gunther disease. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. The manifestations of CEP are heterogeneous, ranging from nonimmune hydrops fetalis due to severe hemolytic anemia in utero to milder, later onset forms, which have only skin lesions due to cutaneous photosensitivity in adult life. The deficiency in UROS activity results in the non-enzymatic conversion of hydroxymethylbilane (HMB) into the uroporphyrinogen-I isomer. Severe congenital erythropoietic porphyria is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non- immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders. Belongs to the uroporphyrinogen-III synthase family.
UniProt Protein Details:

Protein type:EC 4.2.1.75; Mitochondrial; Lyase; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll

Chromosomal Location of Human Ortholog: 10q25.2-q26.3

Cellular Component: cytosol; mitochondrion

Molecular Function:cofactor binding; uroporphyrinogen-III synthase activity

Biological Process: heme biosynthetic process; porphyrin metabolic process; protoporphyrinogen IX biosynthetic process; response to antibiotic; uroporphyrinogen III biosynthetic process

Disease: Porphyria, Congenital Erythropoietic

NCBI Summary:The protein encoded by this gene catalyzes the fourth step of porphyrin biosynthesis in the heme biosynthetic pathway. Defects in this gene cause congenital erythropoietic porphyria (Gunther's disease). [provided by RefSeq, Jul 2008]
UniProt Code:P10746
NCBI GenInfo Identifier:4557873
NCBI Gene ID:7390
NCBI Accession:NP_000366.1
UniProt Secondary Accession:P10746,Q9H2T1, B2RC13, D3DRF7,
UniProt Related Accession:P10746
Molecular Weight:28,628 Da
NCBI Full Name:uroporphyrinogen-III synthase
NCBI Synonym Full Names:uroporphyrinogen III synthase
NCBI Official Symbol:UROS  
NCBI Official Synonym Symbols:UROIIIS  
NCBI Protein Information:uroporphyrinogen-III synthase
UniProt Protein Name:Uroporphyrinogen-III synthase
UniProt Synonym Protein Names:Hydroxymethylbilane hydrolyase [cyclizing]; Uroporphyrinogen-III cosynthase
Protein Family:Uroporphyrinogen-III synthase
UniProt Gene Name:UROS  
UniProt Entry Name:HEM4_HUMAN
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