Overview
Product nameHMBS protein (His tag)See all HMBS proteins and peptides ...
Protein descriptionRecombinant full length Human HMBS (amino acids 1-361) with N terminal His tag; 385 aa, 41.9 kDa inclusive of tag. (NP_000181).
Uniprot accessionP08397
Molecular weight41.900kDa inclusive of tags
Protein length361 amino acids
Expression hostE. coli
Properties
Purity> 95
% by SDS-PAGE
Purification notesab123176 is purified using conventional chromatography techniques.
Mass spectrometryMALDI-TOF
E.C. Number2.5.1.61
FormLiquid
Storage instructionsStore at +4°C short term (1-2 weeks). Aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage bufferpH: 8.00
Constituents: 0.02% DTT, 0.32% Tris HCl, 10% Glycerol, 0.58% Sodium chloride
Concentration information loading...
Sequence notesMGSSHHHHHH 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
Research Areas
Applications
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.
|
Application
|
Notes |
| MS |
MS: Use at an assay dependent concentration. |
| SDS-PAGE |
SDS-PAGE: Use at an assay dependent concentration. |
Protein info
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Alternative names
HEM3_HUMANHMBSHydroxymethylbilane synthase
PBG DPBG-DPBGDPorphobilinogen deaminasePre uroporphyrinogen synthasePre-uroporphyrinogen synthaseUPSUroporphyrinogen I synthaseUroporphyrinogen I synthetase
see all
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.
HMBS protein (His tag) images
References for HMBS protein (His tag) (ab123176)
ab123176
has not yet been referenced specifically in any publications.
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"
SDS-PAGE - HMBS protein (ab123176)15% SDS-PAGE analysis of ab123176 (3ug)