Human ALAD Recombinant Protein (RPPB5801)
- SKU:
- RPPB5801
- Product Type:
- Recombinant Protein
- Species:
- Human
- Uniprot:
- P13716
- Research Area:
- Enzymes
Description
| Product Name: | Human ALAD Recombinant Protein |
| Product Code: | RPPB5801 |
| Size: | 10µg |
| Species: | Human |
| Target: | ALAD |
| Synonyms: | Aminolevulinate delta-dehydratase, ALADH, PBGS, Porphobilinogen synthase, delta-aminolevulinic acid dehydratase, EC 4.2.1.24. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered colorless solution. |
| Formulation: | The ALAD solution (1mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 100mM NaCl and 10% glycerol. |
| Stability: | Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles. |
| Purity: | Greater than 85% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MGSSHHHHHH SSGLVPRGSH MGSHMQPQSV LHSGYFHPLL RAWQTATTTL NASNLIYPIF VTDVPDDIQP ITSLPGVARY GVKRLEEMLR PLVEEGLRCV LIFGVPSRVP KDERGSAADS EESPAIEAIH LLRKTFPNLL VACDVCLCPY TSHGHCGLLS ENGAFRAEES RQRLAEVALA YAKAGCQVVA PSDMMDGRVE AIKEALMAHG LGNRVSVMSY SAKFASCFYG PFRDAAKSSP AFGDRRCYQL PPGARGLALR AVDRDVREGA DMLMVKPGMP YLDIVREVKD KHPDLPLAVY HVSGEFAMLW HGAQAGAFDL KAAVLEAMTA FRRAGADIII TYYTPQLLQW LKEE |
ALAD form porphobilinogen (a precursor of heme, cytochromes and other hemoproteins) by catalyzing the compression of 2 molecules of delta-aminolevulinate. ALAD catalyzes the second step in the porphyrin and heme biosynthetic pathway; zinc is vital for enzymatic activity. ALAD has 8 identical subunits and its enzymatic activity is inhibited by lead. Mutations in the ALAD structural gene are the source for high sensitivity to lead poisoning and acute hepatic porphyria.
ALAD Human Recombinant produced in�E. coli is a single polypeptide chain containing 354 amino acids (1-330) and having a molecular mass of 38.8kDa.ALAD is fused to a 24 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.
| UniProt Protein Function: | ALAD: Catalyzes an early step in the biosynthesis of tetrapyrroles. Binds two molecules of 5-aminolevulinate per subunit, each at a distinct site, and catalyzes their condensation to form porphobilinogen. Defects in ALAD are the cause of acute hepatic porphyria (AHEPP). A form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AHP is characterized by attacks of gastrointestinal disturbances, abdominal colic, paralysis, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors. Belongs to the ALADH family. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; EC 4.2.1.24; Lyase Chromosomal Location of Human Ortholog: 9q32 Cellular Component: cytosol; extracellular region; nucleus Molecular Function:catalytic activity; identical protein binding; porphobilinogen synthase activity; zinc ion binding Biological Process: heme biosynthetic process; neutrophil degranulation; protein homooligomerization Disease: Porphyria, Acute Hepatic |
| NCBI Summary: | The ALAD enzyme is composed of 8 identical subunits and catalyzes the condensation of 2 molecules of delta-aminolevulinate to form porphobilinogen (a precursor of heme, cytochromes and other hemoproteins). ALAD catalyzes the second step in the porphyrin and heme biosynthetic pathway; zinc is essential for enzymatic activity. ALAD enzymatic activity is inhibited by lead and a defect in the ALAD structural gene can cause increased sensitivity to lead poisoning and acute hepatic porphyria. Alternative splicing of this gene results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Dec 2015] |
| UniProt Code: | P13716 |
| NCBI GenInfo Identifier: | 122833 |
| NCBI Gene ID: | 210 |
| NCBI Accession: | P13716.1 |
| UniProt Secondary Accession: | P13716,Q16870, Q16871, Q9BVQ9, A8K375, B2R6F2, |
| UniProt Related Accession: | P13716 |
| Molecular Weight: | 36kDa |
| NCBI Full Name: | Delta-aminolevulinic acid dehydratase |
| NCBI Synonym Full Names: | aminolevulinate dehydratase |
| NCBI Official Symbol: | ALAD�� |
| NCBI Official Synonym Symbols: | PBGS; ALADH�� |
| NCBI Protein Information: | delta-aminolevulinic acid dehydratase |
| UniProt Protein Name: | Delta-aminolevulinic acid dehydratase |
| UniProt Synonym Protein Names: | Porphobilinogen synthase |
| Protein Family: | Aladin |
| UniProt Gene Name: | ALAD�� |
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