Mouse Porphobilinogen deaminase, HMBS ELISA Kit | HMBS elisa kit
Mouse Porphobilinogen deaminase, HMBS ELISA Kit
NCBI and Uniprot Product Information
Uniprot Description
HMBS: Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps. Defects in HMBS are the cause of acute intermittent porphyria (AIP). AIP is 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. AIP is an autosomal dominant form of hepatic porphyria characterized by acute attacks of neurological dysfunctions with abdominal pain, hypertension, tachycardia, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors. Belongs to the HMBS family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Mitochondrial; EC 2.5.1.61; Transferase; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll
Cellular Component: axon; cytoplasm; condensed chromosome; nucleus
Molecular Function: transferase activity; amine binding; carboxylic acid binding; uroporphyrinogen-III synthase activity; coenzyme binding; hydroxymethylbilane synthase activity
Biological Process: tetrapyrrole biosynthetic process; peptidyl-pyrromethane cofactor linkage; porphyrin biosynthetic process; heme biosynthetic process
Research Articles on HMBS
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Product Notes
The Mouse HMBS hmbs (Catalog #AAA9329275) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9329275 ELISA Kit recognizes Mouse HMBS. It is sometimes possible for the material contained within the vial of "Porphobilinogen deaminase, HMBS ELISA Kit, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
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