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HMBS Antibody / Hydroxymethylbilane synthase

CAT: 0800-RQ4407Size: 100 µgDry Ice: NoHazardous: No
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CAT#:0800-RQ4407Size:100 µg
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Description
Porphobilinogen deaminase (hydroxymethylbilane synthase, or uroporphyrinogen I synthase) is an enzyme that in humans is encoded by the HMBS gene. 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.
CAS Number
9007-83-4
Specifications
Western blot: 0.5-1 µg/mL, Direct ELISA: 0.1-0.5 µg/mL (recombinant human protein) (BSA-free format available)
UniProt
P08397
Host
Rabbit
Reactivity
Human, Mouse, Rat
Immunogen
A human partial recombinant protein corresponding to amino acids N6-H361 was used as the immunogen for the HMBS antibody.
Clonality
Polyclonal
Isotype
IgG
Applications
WB, ELISA
Purity
Antigen affinity purified
Format
Antigen affinity purified
Buffer
Lyophilized from 1X PBS with 2% Trehalose
Reconstitution
After reconstitution, the HMBS antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.
Limitations
This HMBS antibody is available for research use only.
Storage Conditions
After reconstitution, the HMBS antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.
Formulation
0.5 mg/mL if reconstituted with 0.2ml sterile DI water
Applications Notes
Optimal dilution of the HMBS antibody should be determined by the researcher.
Image Legend
Western blot testing of human 1) HeLa, 2) placenta, 3) HepG2, 4) 22RV1 and 5) U-2 OS cell lysate with HMBS antibody at 0.5ug/ml. Predicted molecular weight: ~39 kDa.

UniProtKB · P08397

Porphobilinogen deaminase

HEM3_HUMAN · Homo sapiens

View on UniProt ↗
Primary accession
P08397
Review status
UniProtKB reviewed (Swiss-Prot)
Gene
HMBS
Protein existence
1: Evidence at protein level
Organism
Homo sapiens (Human)
Taxonomy ID
9606
Alternative names
PBG-D
EC number
2.5.1.61
Processing
—
Secondary accessions
A8K2L0, G3V1P4, G5EA58, P08396, Q16012
Protein keywords

Technical term

3D-structureDirect protein sequencingProteomics identificationReference proteome

PTM

AcetylationPhosphoprotein

Coding sequence diversity

Alternative splicing

Cellular component

Cytoplasm

Disease

Disease variant

Biological process

Heme biosynthesisPorphyrin biosynthesis

Molecular function

Transferase

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