HMBS Recombinant Rabbit Monoclonal Antibody [SC65-07]
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.
Product Specifications
CAS Number
9000-83-3
Product Name Alternative
Abbreviation
PBG-D, HMBS
Swiss Prot
P08397 Human, P22907 Mouse, P19356 Rat
Cellular Locus
Cytoplasm.
Host
Rabbit
Species Reactivity
Human,Mouse,Rat
Immunogen
Recombinant protein within Human HMBS aa 1-132 / 299.
Isotype
IgG
Conjugation
Non-conjugated
Type
Recombinant Rabbit monoclonal Antibody
Applications
WB
Positive Control
Hela cell lysate, 293T cell lysate, K562 cell lysates.
Concentration
1 mg/mL
Dilution
WB: 1:500
Purity
Protein A affinity purified.
Form
Liquid
Buffer
1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
Molecular Weight
Predicted band size: 39 kDa
Storage Conditions
Store at +4°C after thawing. Aliquot store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Recombinant Antibody
Yes
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