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HMBS
Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps. Belongs to the HMBS family. Isoform 1 is ubiquitously expressed. Isoform 2 is found only in erythroid cells. 4 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
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| Protein type: Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; EC 2.5.1.61; Mitochondrial; Transferase |
| Chromosomal Location of mouse Ortholog: 9 A5.2|9 24.84 cM |
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Cellular Component:
axon; condensed chromosome; cytoplasm; perinuclear region of cytoplasm
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Molecular Function:
amine binding; carboxylic acid binding; hydroxymethylbilane synthase activity; transferase activity; uroporphyrinogen-III synthase activity
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Biological Process:
heme biosynthetic process; liver development; peptidyl-pyrromethane cofactor linkage; porphyrin-containing compound biosynthetic process; porphyrin-containing compound metabolic process; response to xenobiotic stimulus; tetrapyrrole biosynthetic process
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Reference #:
P22907
(UniProtKB)
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Alt. Names/Synonyms: HEM3; Hmbs; hydroxymethylbilane synthase; PBG; PBG-D; PBGD; Porphobilinogen deaminase; pre-uroporphyrinogen synthase; T25658; Ups; Uros1
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Gene Symbols: Hmbs
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Molecular weight:
39,344 Da
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Basal Isoelectric point:
6.41
Predict pI for various phosphorylation states
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