Human HMBS full length protein (ab123176)
- Product nameHuman HMBS full length proteinSee all HMBS proteins and peptides ...
- DescriptionRecombinant, His tag
- SourceE. coli
- Amino Acid Sequence
- SequenceMGSSHHHHHH SSGLVPRGSH MGSHMSGNGN AAATAEENSP KMRVIRVGTR KSQLARIQTD SVVATLKASY PGLQFEIIAM STTGDKILDT ALSKIGEKSL FTKELEHALE KNEVDLVVHS LKDLPTVLPP GFTIGAICKR ENPHDAVVFH PKFVGKTLET LPEKSVVGTS SLRRAAQLQR KFPHLEFRSI RGNLNTRLRK LDEQQEFSAI ILATAGLQRM GWHNRVGQIL HPEECMYAVG QGALGVEVRA KDQDILDLVG VLHDPETLLR CIAERAFLRH LEGGCSVPVA VHTAMKDGQL YLTGGVWSLD GSDSIQETMQ ATIHVPAQHE DGPEDDPQLV GITARNIPRG PQLAAQNLGI SLANLLLSKG AKNILDVARQ LNDAH
- Molecular weight42 kDa including tags
- Amino acids1 to 361
- TagsHis tag N-Terminus
Our Abpromise guarantee covers the use of ab123176 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
- Mass spectrometryMALDI-TOF
- Purity> 95
% by SDS-PAGE.
ab123176 is purified using conventional chromatography techniques.
- Concentration information loading...
Preparation and Storage
- Stability and Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Constituents: 0.02% DTT, 0.32% Tris HCl, 10% Glycerol, 0.58% Sodium chloride
- Hydroxymethylbilane synthase
- PBG D
- Porphobilinogen deaminase
- Pre uroporphyrinogen synthase
- Pre-uroporphyrinogen synthase
- Uroporphyrinogen I synthase
- Uroporphyrinogen I synthetase
- FunctionTetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.
- Tissue specificityIsoform 1 is ubiquitously expressed. Isoform 2 is found only in erythroid cells.
- PathwayPorphyrin metabolism; protoporphyrin-IX biosynthesis; coproporphyrinogen-III from 5-aminolevulinate: step 2/4.
- Involvement in diseaseDefects in HMBS are the cause of acute intermittent porphyria (AIP) [MIM:176000]. 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.
- Sequence similaritiesBelongs to the HMBS family.
- Cellular localizationCytoplasm.
References for Human HMBS full length protein (ab123176)
ab123176 has not yet been referenced specifically in any publications.