HMBS Monoclonal Antibody (OTI2F4), TrueMAB™, OriGene

Mouse Monoclonal Antibody

Supplier:  OriGene TA802521

Catalog No. 50-167-9286

Description

PBGD (porphobilinogen deaminase), also designated hydroxymethylbilane synthase, is a cytoplasmic enzyme found in the heme synthesis pathway. PBGD belongs to the HMBS (hydroxymethylbilane synthase) family. Deficiency of PBGD causes errors in pyrrole metabolism, which in turn leads to an inherited autosomal disorder called acute intermittent porphyria (AIP). AIP is characterized by acute attacks of neurological dysfunctions with hypertension, tachycardia, peripheral neurologic disturbances, abdominal pain and excessive amounts of aminolevulinic acid and porphobilinogen in the urine.

Specifications

• Antigen: HMBS
• Classification: Monoclonal
• Concentration: 1 mg/mL
• Formulation: PBS with 1% BSA, 50% glycerol and 0.02% sodium azide
• Gene Accession No.: P08397
• Gene Symbols: HMBS
• Immunogen: Full length human recombit protein of human HMBS produced in HEK293T cell.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG1

• Applications: Western Blot
• Clone: OTI2F4
• Conjugate: Unconjugated
• Gene: HMBS
• Gene Alias: PBG-D, PBGD, PORC, UPS
• Host Species: Mouse
• Purification Method: Affinity Chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 3145
• Content And Storage: -20° C, Avoid Freeze/Thaw Cycles
• Form: Liquid