Full Product Name
HMBS Rabbit Polyclonal
Product Gene Name
anti-HMBS antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Species Reactivity
Human, Mouse, Rat
Purity/Purification
>=95% as determined by SDS-PAGE
Immunogen Affinity Purified
Immunogen
Hydroxymethylbilane synthase
Preparation and Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of anti-HMBS antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Applications Tested/Suitable for anti-HMBS antibody
ELISA (EIA), Western Blot (WB), Immunofluorescence (IF)
NCBI/Uniprot data below describe general gene information for HMBS. It may not necessarily be applicable to this product.
NCBI Accession #
AKI70033.1
[Other Products]
UniProt Secondary Accession #
P08396; Q16012; A8K2L0; G3V1P4; G5EA58[Other Products]
UniProt Related Accession #
P08397[Other Products]
Molecular Weight
33,264 Da
NCBI Official Full Name
HMBS, partial
NCBI Official Synonym Full Names
hydroxymethylbilane synthase
NCBI Official Symbol
HMBS [Similar Products]
NCBI Official Synonym Symbols
UPS; PBGD; PORC; PBG-D
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NCBI Protein Information
porphobilinogen deaminase
UniProt Protein Name
Porphobilinogen deaminase
UniProt Synonym Protein Names
Hydroxymethylbilane synthase; HMBS; Pre-uroporphyrinogen synthase
UniProt Gene Name
HMBS [Similar Products]
UniProt Synonym Gene Names
PBGD; UPS; PBG-D; HMBS [Similar Products]
UniProt Entry Name
HEM3_HUMAN
NCBI Summary for HMBS
This gene encodes a member of the hydroxymethylbilane synthase superfamily. The encoded protein is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2008]
UniProt Comments for HMBS
HMBS: Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps. Defects in HMBS are the cause of acute intermittent porphyria (AIP). AIP is a form of porphyria. 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. AIP is an autosomal dominant form of hepatic porphyria characterized by acute attacks of neurological dysfunctions with abdominal pain, hypertension, tachycardia, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors. Belongs to the HMBS family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Mitochondrial; EC 2.5.1.61; Transferase
Chromosomal Location of Human Ortholog: 11q23.3
Cellular Component: axon; condensed chromosome; cytosol; nucleus
Molecular Function: amine binding; carboxylic acid binding; coenzyme binding; hydroxymethylbilane synthase activity; uroporphyrinogen-III synthase activity
Biological Process: astrocyte differentiation; heme biosynthetic process; organ regeneration; peptidyl-pyrromethane cofactor linkage; protoporphyrinogen IX biosynthetic process; response to amino acid stimulus; response to carbohydrate stimulus; response to cobalt ion; response to drug; response to estradiol stimulus; response to hypoxia; response to methylmercury; response to vitamin; response to zinc ion
Disease: Porphyria, Acute Intermittent
Research Articles on HMBS
1. ALAS1 mRNA and activity were elevated approximately ~3- and 5-fold, and HMB synthase activity was approximately half-normal (~42%)
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