HMBS Mouse anti-Human, Clone: OTI3E2, lyophilized, TrueMAB™ 

Mouse Monoclonal Antibody

Manufacturer:  OriGene CF802728

Catalog No. 50-166-6125

Description

Reconstitute with PBS (pH 7.3) and recommend to perform another round of desalting process using Product No. 7KMWCO

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
• Conjugate: Unconjugated
• Gene: HMBS
• Format: Lyophilized
• Gene Symbols: HMBS
• Immunogen: Full length human recombit protein of human HMBS produced in HEK293T cell.
• Purification Method: Affinity Chromatography
• Regulatory Status: RUO
• Type: Antibody
• Monoclonal or Polyclonal: Monoclonal
• Target Species: Human

• Applications: Western Blot
• Clone: OTI3E2
• Formulation: PBS with 8% trehalose and no preservative; pH 7.3
• Gene Accession No.: P08397
• Gene Alias: PBG-D, PBGD, PORC, UPS
• Host Species: Mouse
• Isotype: IgG1
• Quantity: 100 μg
• Storage Requirements: -20° C, Avoid Freeze/Thaw Cycles
• Primary or Secondary: Primary
• Gene ID (Entrez): 3145