产品名称
HEM6 Rabbit Polyclonal Antibody
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
https://www.uniprot.org/uniprot/1371
Human Swissprot No.
P36551
Human Swissprot Link
https://www.uniprot.org/uniprotkb/P36551/entry
Mouse Gene Link
https://www.uniprot.org/uniprot/12892
Mouse Swissprot No.
P36552
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/P36552
Rat Gene Link
https://www.uniprot.org/uniprot/304024
Rat Swissprot Link
https://www.uniprot.org/uniprotkb/Q3B7D0
免疫原
Synthesized peptide derived from human HEM6 AA range: 265-315
特异性
This antibody detects endogenous levels of HEM6 at Human/Mouse/Rat
宿主
Polyclonal, Rabbit,IgG
背景介绍
The protein encoded by this gene is the sixth enzyme of the heme biosynthetic pathway. The encoded enzyme is soluble and found in the intermembrane space of mitochondria. This enzyme catalyzes the stepwise oxidative decarboxylation of coproporphyrinogen III to protoporphyrinogen IX, a precursor of heme. Defects in this gene are a cause of hereditary coproporphyria (HCP).[provided by RefSeq, Oct 2009],
细胞定位
Mitochondrion intermembrane space.
功能
catalytic activity:Coproporphyrinogen-III + O(2) + 2 H(+) = protoporphyrinogen-IX + 2 CO(2) + 2 H(2)O.,disease:Defects in CPOX are the cause of hereditary coproporphyria (HCP) [MIM:121300]. HCP is an acute hepatic porphyria and an autosomal dominant disease characterized by neuropsychiatric disturbances and skin photosensitivity. Biochemically, there is an overexcretion of coproporphyrin III in the urine and in the feces. HCP is clinically characterized by attacks of abdominal pain, neurological disturbances, and psychiatric symptoms. The symptoms are generally manifested with rapid onset, and can be precipitated by drugs, alcohol, caloric deprivation, infection, endocrine factors or stress. A severe variant form is harderoporphyria, which is characterized by earlier onset attacks, massive excretion of harderoporphyrin in the feces, and a marked decrease of coproporphyrinogen IX oxidase activity.,pathway:Porphyrin metabolism; protoporphyrin-IX biosynthesis; protoporphyrinogen-IX from coproporphyrinogen-III (O2 route): step 1/1.,similarity:Belongs to the aerobic coproporphyrinogen-III oxidase family.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
