Rabbit HMBS Monoclonal Antibody | anti-HMBS antibody
HMBS Conjugated Antibody
AF350 conjugated: most applications: 1:50-1:250
AF405 conjugated: most applications: 1:50-1:250
AF488 conjugated: most applications: 1:50-1:250
AF555 conjugated: most applications: 1:50-1:250
AF594 conjugated: most applications: 1:50-1:250
AF647 conjugated: most applications: 1:50-1:250
AF680 conjugated: most applications: 1:50-1:250
AF750 conjugated: most applications: 1:50-1:250
Biotin conjugated: working with enzyme-conjugated streptavidin, most applications: 1:50-1:1000
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a member of the hydroxymethylbilane synthase superfamily. The encoded protein is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2008]
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: EC 2.5.1.61; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Transferase; Mitochondrial
Chromosomal Location of Human Ortholog: 11q23.3
Cellular Component: cytosol
Molecular Function: hydroxymethylbilane synthase activity
Biological Process: porphyrin metabolic process; protoporphyrinogen IX biosynthetic process; peptidyl-pyrromethane cofactor linkage; heme biosynthetic process
Disease: Porphyria, Acute Intermittent
Research Articles on HMBS
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Product Notes
The HMBS hmbs (Catalog #AAA9456737) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The HMBS Conjugated Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's HMBS can be used in a range of immunoassay formats including, but not limited to, Most Applications. Suggested Dilution: AF350 conjugated: most applications: 1:50-1:250 AF405 conjugated: most applications: 1:50-1:250 AF488 conjugated: most applications: 1:50-1:250 AF555 conjugated: most applications: 1:50-1:250 AF594 conjugated: most applications: 1:50-1:250 AF647 conjugated: most applications: 1:50-1:250 AF680 conjugated: most applications: 1:50-1:250 AF750 conjugated: most applications: 1:50-1:250 Biotin conjugated: working with enzyme-conjugated streptavidin, most applications: 1:50-1:1000. Researchers should empirically determine the suitability of the HMBS hmbs for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "HMBS, Monoclonal Antibody" 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
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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