Metabolism Antibodies 3
Anti-HMBS Antibody (CAB11701)
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- SKU:
- CAB11701
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Monoclonal Antibody
- Research Area:
- Metabolism
Description
| Antibody Name: | Anti-HMBS Antibody |
| Antibody SKU: | CAB11701 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | A synthesized peptide derived from human HMBS |
| Application: | WB |
| Recommended Dilution: | WB 1:500 - 1:2000 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | Mouse kidney, Mouse heart, HepG2, Rat kidney |
| Immunogen: | A synthesized peptide derived from human HMBS |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | Email for sequence |
| Gene ID: | 3145 |
| Uniprot: | P08397 |
| Cellular Location: | |
| Calculated MW: | 42kDa |
| Observed MW: | 42KDa |
 | Western blot - HMBS Rabbit mAb (CAB11701) |
| Synonyms: | PBG-D, PBGD, PORC, UPS |
| Background: | 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 Protein Function: | 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. |
| UniProt Protein Details: | Protein type:EC 2.5.1.61; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Transferase; Mitochondrial Chromosomal Location of Human Ortholog: 11q23.3 Cellular Component: cytosol Molecular Function:hydroxymethylbilane synthase activity Biological Process: porphyrin metabolic process; protoporphyrinogen IX biosynthetic process; peptidyl-pyrromethane cofactor linkage; heme biosynthetic process Disease: Porphyria, Acute Intermittent |
| NCBI Summary: | 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 Code: | P08397 |
| NCBI GenInfo Identifier: | 1170217 |
| NCBI Gene ID: | 3145 |
| NCBI Accession: | P08397.2 |
| UniProt Related Accession: | P08397 |
| Molecular Weight: | 39kDa |
| NCBI Full Name: | Porphobilinogen deaminase |
| NCBI 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 Synonym Protein Names: | Hydroxymethylbilane synthase; HMBS; Pre-uroporphyrinogen synthase |
| UniProt Gene Name: | HMBS |
| UniProt Entry Name: | HEM3_HUMAN |
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system_update_altDatasheetHuman HMBS Recombinant Protein Porphobilinogen Deaminase (HMBS) is a member of the HMBS family. PBGD is the third enzyme of the heme biosynthetic pathway and catalyzes t