Anti-HMBS Antibody (CAB1777)
- SKU:
- CAB1777
- Product type:
- Antibody
- Reactivity:
- Human
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Frequently bought together:
Description
| Antibody Name: | Anti-HMBS Antibody |
| Antibody SKU: | CAB1777 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB |
| Reactivity: | Human, Mouse |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-361 of human HMBS (NP_000181.2). |
| Application: | WB |
| Recommended Dilution: | WB 1:500 - 1:2000 |
| Reactivity: | Human, Mouse |
| Positive Samples: | HeLa, Raji, Mouse spleen |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-361 of human HMBS (NP_000181.2). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | MSGN GNAA ATAE ENSP KMRV IRVG TRKS QLAR IQTD SVVA TLKA SYPG LQFE IIAM STTG DKIL DTAL SKIG EKSL FTKE LEHA LEKN EVDL VVHS LKDL PTVL PPGF TIGA ICKR ENPH DAVV FHPK FVGK TLET LPEK SVVG TSSL RRAA QLQR KFPH LEFR SIRG NLNT RLRK LDEQ QEFS AIIL ATAG LQRM GWHN RVGQ ILHP EECM YAVG QGAL GVEV RAKD QDIL DLVG VLHD PETL LRCI AERA FLRH LEGG CSVP VAVH TAMK DGQL YLTG GVWS LDGS DSIQ ETMQ ATIH VPAQ HEDG PEDD PQLV GITA RNIP RGPQ LAAQ NLGI SLAN LLLS KGAK NILD VARQ LNDA H |
| Gene ID: | 3145 |
| Uniprot: | P08397 |
| Cellular Location: | Cytoplasm |
| Calculated MW: | 33kDa/34kDa/37kDa/39kDa |
| Observed MW: | 45kDa |
| Synonyms: | HMBS, 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. |
| 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 |
 | Western blot analysis of extracts of various cell lines, using HMBS antibody (CAB1777) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 5s. |
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