HMBS Mouse anti-Human, Clone: OTI1F1, Invitrogen
Mouse Monoclonal Antibody
Manufacturer: Invitrogen MA526541
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.
|PBS with 1% BSA, 50% glycerol and 0.02% sodium azide; pH 7.3|
|HEM3, HMBS, Hydroxymethylbilane synthase, PBG D, PBG-D, PBGD, Porphobilinogen deaminase, Pre uroporphyrinogen synthase, Pre-uroporphyrinogen synthase, UPS, Uroporphyrinogen I synthase, Uroporphyrinogen I synthetase|
|-20° C, Avoid Freeze/Thaw Cycles|
|Full length human recombit protein of HMBS produced in HEK293T cell|
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