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Item number | Size | Datasheet | Manual | SDS | Delivery time | Quantity | Price |
---|---|---|---|---|---|---|---|
045433.100 | 100 µg | - | - |
3 - 19 business days* |
568.00€
|
||
045433.500 | 500 µg | - | - |
3 - 19 business days* |
1,099.00€
|
If you have any questions, please use our Contact Form.
You can also order by e-mail: info@biomol.com
Larger quantity required? Request bulk
You can also order by e-mail: info@biomol.com
Larger quantity required? Request bulk
Porphobilinogen deaminase, also known as HMBS, is a member of the hydroxymethylbilane synthase... more
Product information "HMBS, Recombinant, Human, aa1-361, His-Tag (Porphobilinogen Deaminase, PBG-D, Hydroxymethylbilane Sy"
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. Source: Recombinant corresponding to aa1-361 from human HMBS, fused to His-tag at N-terminus, expressed in E.coli. Molecular Weight: ~41.9kD (385aa) confirmed by MALDI-TOF, AA Sequence: MGSSHHHHHH SSGLVPRGSH MGSHMSGNGN AAATAEENSP KMRVIRVGTR KSQLARIQTD SVVATLKASY PGLQFEIIAM STTGDKILDT ALSKIGEKSL FTKELEHALE KNEVDLVVHS LKDLPTVLPP GFTIGAICKR ENPHDAVVFH PKFVGKTLET LPEKSVVGTS SLRRAAQLQR KFPHLEFRSI RGNLNTRLRK LDEQQEFSAI ILATAGLQRM GWHNRVGQIL HPEECMYAVG QGALGVEVRA KDQDILDLVG VLHDPETLLR CIAERAFLRH LEGGCSVPVA VHTAMKDGQL YLTGGVWSLD GSDSIQETMQ ATIHVPAQHE DGPEDDPQLV GITARNIPRG PQLAAQNLGI SLANLLLSKG AKNILDVARQ LNDAH, Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 6 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
Keywords: | PBGD, HMBS, PBG-D, EC=2.5.1.61, Porphobilinogen deaminase, Hydroxymethylbilane synthase, Pre-uroporphyrinogen synthase |
Supplier: | United States Biological |
Supplier-Nr: | 045433 |
Properties
Conjugate: | No |
Species reactivity: | human |
Format: | Highly Purified |
Database Information
KEGG ID : | K01749 | Matching products |
UniProt ID : | P08397 | Matching products |
Gene ID | GeneID 3145 | Matching products |
Handling & Safety
Storage: | -20°C |
Shipping: | +4°C (International: +4°C) |
Caution
Our products are for laboratory research use only: Not for administration to humans!
Our products are for laboratory research use only: Not for administration to humans!
Information about the product reference will follow.
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