Rat CAAX prenyl protease 1 homolog (ZMPSTE24) ELISA Kit | ZMPSTE24 elisa kit
Rat CAAX prenyl protease 1 homolog (ZMPSTE24) ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a member of the peptidase M48A family. The encoded protein is a zinc metalloproteinase involved in the two step post-translational proteolytic cleavage of carboxy terminal residues of farnesylated prelamin A to form mature lamin A. Mutations in this gene have been associated with mandibuloacral dysplasia and restrictive dermopathy. [provided by RefSeq, Jul 2008]
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: Membrane protein, multi-pass; Protease; Membrane protein, integral; EC 3.4.24.84
Chromosomal Location of Human Ortholog: 1p34
Cellular Component: endoplasmic reticulum membrane; membrane; integral to membrane; nuclear inner membrane
Molecular Function: metalloexopeptidase activity; metal ion binding; metalloendopeptidase activity
Biological Process: prenylated protein catabolic process; proteolysis; nuclear membrane organization and biogenesis
Disease: Restrictive Dermopathy, Lethal; Mandibuloacral Dysplasia With Type B Lipodystrophy
Research Articles on ZMPSTE24
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Product Notes
The Rat ZMPSTE24 zmpste24 (Catalog #AAA7232008) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA7232008 ELISA Kit recognizes Rat ZMPSTE24. It is sometimes possible for the material contained within the vial of "CAAX prenyl protease 1 homolog (ZMPSTE24), 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
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