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

Polyclonal

Rabbit

Affinity Purified

Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

For short term use, store at 2-8 degree C up to 1 week. For long term storage, store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-ZMPSTE24 antibody 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.

This is a rabbit polyclonal antibody against ZMPSTE24. It was validated on Western Blot using a cell lysate as a positive control.

Target Description: ZMPSTE24 is a member of the peptidase M48A family. This 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 ZMPSTE24 gene have been associated with mandibuloacral dysplasia and restrictive dermopathy.The protein encoded by this gene is a zinc metalloprotease similar to yeast Ste24p. It is an integral membrane protein belonging to peptidase family M48 and is found in the endoplasmic reticulum and possibly in the Golgi compartment. It is thought to be involved in the proteolytic processing of farnesylated proteins.

Polyclonal; Signal Proteins; Membrane Protein; Drugs and Drug Metabolism; Disease Related;

Western Blot (WB)

WB Suggested Anti-ZMPSTE24 Antibody Titration: 0.2-1 ug/ml
Positive Control: Jurkat cell lysate

55kDa

zinc metallopeptidase STE24

CAAX prenyl protease 1 homolog

CAAX prenyl protease 1 homolog

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

FACE1_HUMAN

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]

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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