Product Description for HMBS
HMBS.
Presentation: Purified
Presentation: Purified
Properties for HMBS
| Product Category | Proteins & Growth Factors |
|---|---|
| Quantity | 0.5 mg |
| Synonyms | Hydroxymethylbilane synthase, PBGD, Porphobilinogen deaminase, Pre-uroporphyrinogen synthase, UPS |
| Presentation | Purified |
| Molecular weight | 41.9 kDa |
| Source | E. coli |
| Species (Protein) | Human |
| Shipping to | Worldwide |
| PDF datasheet | View Datasheet |
| Manufacturer | OriGene Technologies GmbH |
| Material safety datasheet | MSDS for Proteins (de) |
Datasheet Extract
| Immunogen |
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|---|---|
| Purity | > 95 % by SDS - PAGE |
| Background | Porphobilinogen deaminase, also known as HMBS, is a member of the hydroxymethylbilane synthase superfamily. It is a cytoplasmic enzyme found in the heme synthesis pathway. Deficiency of HMBS causes errors in pyrrole metabolism which in turn leads to an inherited autosomal disorder called acute intermittent porphyria (AIP) which 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. |
| Concentration | 1 mg/ml (determined by Bradford assay) |
| General Readings | Schneider Yin X., et al. (2004) J Inherit Metab Dis. 625-631:471-474.Sheppard L., et al. (1995) Paediatr Anaesth. 15:426-428. |
| Storage | Store undiluted at 2-8°C for one week or (in aliquots) at -20°C to -80°C for longer. Avoid repeated freezing and thawing. Shelf life: one year from despatch. |
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