Rabbit HMBS Polyclonal Antibody | anti-HMBS antibody

Anti-HMBS Antibody

Cat. Num. AAA419814

• Gene Names: HMBS; UPS; PBGD; PORC; PBG-D
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry
• Purity: Affinity Purification
• Synonyms: HMBS; Polyclonal Antibody; Anti-HMBS Antibody; anti-HMBS antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Western blot analysis of extracts of various cell lines, using HMBS antibody. Immunohistochemistry of paraffin-embedded human stomach using HMBS antibody at dilution of 1:100 (200x lens). Immunohistochemistry of paraffin-embedded human stomach cancer using HMBS antibody at dilution of 1:100 (200x lens).
• Purity/Purification: Affinity Purification
• Form/Format: Liquid
• Concentration: 1mg/ml (varies by lot)
• Sequence Length: 361

• Applicable Applications for anti-HMBS antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: WB: 1:500 - 1:2000; IHC: 1:50- 1:200
• Immunogen: Recombinant protein of human HMBS
• Storage Buffer: PBS with 0.02% sodium azide,50% glycerol, pH7.3.
• Preparation and Storage: Store at -20 degree C (regular) and -80 degree C (long term). Avoid freeze / thaw cycles.

Western Blot (WB)

Immunohistochemistry (IHC)

Immunohistochemistry (IHC)

Related Product Information for anti-HMBS antibody

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.

NCBI and Uniprot Product Information

• NCBI GI #: 823669642
• NCBI GeneID: 3145
• Molecular Weight: 33,264 Da
• NCBI Official Full Name: HMBS, partial
• NCBI Official Synonym Full Names: hydroxymethylbilane synthase
• NCBI Official Symbol: HMBS
• NCBI Official Synonym Symbols: UPS; PBGD; PORC; PBG-D
• NCBI Protein Information: porphobilinogen deaminase
• UniProt Protein Name: Porphobilinogen deaminase
• UniProt Gene Name: HMBS
• UniProt Synonym Gene Names: PBGD; UPS; PBG-D; HMBS

NCBI Description

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 Description

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; EC 2.5.1.61; Mitochondrial; Transferase

Chromosomal Location of Human Ortholog: 11q23.3

Cellular Component: cytoplasm; cytosol

Molecular Function: hydroxymethylbilane synthase activity

Biological Process: heme biosynthetic process

Disease: Porphyria, Acute Intermittent