CAAX prenyl protease 1 homolog; Farnesylated proteins-converting enzyme 1; FACE-1; Prenyl protein-specific endoprotease 1; Zinc metalloproteinase Ste24 homolog; FACE1; STE24

For Research Use Only. Not for use in diagnostic procedures.

Natural and recombinant Human CAAX prenyl protease 1 homolog

For long term storage, please store the entire kit at -20 degree C.

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Small volumes of ZMPSTE24 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS2889872 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the ZMPSTE24/CAAX Prenyl Protease 1 Homolog, ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing ZMPSTE24. The ELISA analytical biochemical technique of the MBS2889872 kit is based on ZMPSTE24 antibody-ZMPSTE24 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect ZMPSTE24 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, ZMPSTE24. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

zinc metallopeptidase, STE24

CAAX prenyl protease 1 homolog

CAAX prenyl protease 1 homolog

FACE1; STE24; FACE-1??[Similar Products]

FACE1_HUMAN

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, integral; Protease; EC 3.4.24.84; Membrane protein, multi-pass

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

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