Anti-HMBS Antibody [SC65-07]
ET1610-91
ApplicationsWestern Blot
Product group Antibodies
ReactivityHuman
TargetHMBS
Overview
- SupplierHUABIO
- Product NameAnti-HMBS Antibody [SC65-07]
- Delivery Days Customer7
- ApplicationsWestern Blot
- Applications SupplierWB
- CertificationResearch Use Only
- ClonalityMonoclonal
- Clone IDSC65-07
- Concentration1 mg/ml
- ConjugateUnconjugated
- Gene ID3145
- Target nameHMBS
- Target descriptionhydroxymethylbilane synthase
- Target synonymsENCEP, LENCEP, PBG-D, PBGD, PORC, UPS, porphobilinogen deaminase, porphyria, acute; Chester type, pre-uroporphyrinogen synthase, uroporphyrinogen I synthase, uroporphyrinogen I synthetase
- HostRabbit
- IsotypeIgG
- Protein IDP08397
- Protein NamePorphobilinogen deaminase
- Scientific DescriptionPBGD (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.
- ReactivityHuman
- Reactivity SupplierHuman
- Reactivity Supplier NoteMouse,Rat
- Storage Instruction-20°C,2°C to 8°C
- UNSPSC41116161