ZMPSTE24 positive control
ZMPSTE24 Antibody Positive Control
Proteolytically removes the C-terminal three residues of farnesylated proteins. Acts on lamin A/C.
NCBI and Uniprot Product Information
Uniprot Description
ZMPSTE24: Proteolytically removes the C-terminal three residues of farnesylated proteins. Acts on lamin A/C. Defects in ZMPSTE24 are the cause of mandibuloacral dysplasia with type B lipodystrophy (MADB). Mandibuloacral dysplasia (MAD) is a rare autosomal recessive disorder characterized by mandibular and clavicular hypoplasia, acroosteolysis, delayed closure of the cranial suture, joint contractures, and types A or B patterns of lipodystrophy. Type B lipodystrophy observed in MADB, is characterized by generalized fat loss. Defects in ZMPSTE24 are a cause of lethal tight skin contracture syndrome (LTSCS); also called restrictive dermopathy (RD). Lethal tight skin contracture syndrome is a rare disorder mainly characterized by intrauterine growth retardation, tight and rigid skin with erosions, prominent superficial vasculature and epidermal hyperkeratosis, facial features (small mouth, small pinched nose and micrognathia), sparse/absent eyelashes and eyebrows, mineralization defects of the skull, thin dysplastic clavicles, pulmonary hypoplasia, multiple joint contractures and an early neonatal lethal course. Liveborn children usually die within the first week of life. The overall prevalence of consanguineous cases suggested an autosomal recessive inheritance. Belongs to the peptidase M48A family.
Protein type: Protease; Membrane protein, multi-pass; Membrane protein, integral; EC 3.4.24.84
Chromosomal Location of Human Ortholog: 1p34
Cellular Component: endoplasmic reticulum membrane; integral to membrane; membrane; nuclear inner membrane
Molecular Function: metal ion binding; metalloendopeptidase activity; metalloexopeptidase activity
Biological Process: nuclear membrane organization and biogenesis; prenylated protein catabolic process; proteolysis
Disease: Mandibuloacral Dysplasia With Type B Lipodystrophy; Restrictive Dermopathy, Lethal
Research Articles on ZMPSTE24
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Product Notes
The ZMPSTE24 zmpste24 (Catalog #AAA543409) is a Positive Control and is intended for research purposes only. The product is available for immediate purchase. AAA Biotech's ZMPSTE24 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Western Blot: 1:500. Researchers should empirically determine the suitability of the ZMPSTE24 zmpste24 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 "ZMPSTE24, Positive Control" 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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