ZMPSTE24 blocking peptide

ZMPSTE24 Antibody (C-term) Blocking Peptide

• Gene Names: ZMPSTE24; HGPS; PRO1; FACE1; STE24; FACE-1; Ste24p
• Synonyms: ZMPSTE24; ZMPSTE24 Antibody (C-term) Blocking Peptide; CAAX prenyl protease 1 homolog; Farnesylated proteins-converting enzyme 1; FACE-1; Prenyl protein-specific endoprotease 1; Zinc metalloproteinase Ste24 homolog; FACE1; STE24; ZMPSTE24 blocking peptide

• Specificity: The synthetic peptide sequence used to generate the antibody was selected from the C-term region of human ZMPSTE24. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.
• Form/Format: The synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml deionized water for a final concentration of 1 mg/ml.
• Sequence Length: 475

• Cellular Location: Endoplasmic reticulum membrane; Multi-pass membrane protein. Nucleus inner membrane; Multi-pass membrane protein
• Tissue Location: Widely expressed. High levels in kidney, prostate, testis and ovary
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Related Product Information for ZMPSTE24 blocking peptide

Proteolytically removes the C-terminal three residues of farnesylated proteins. Acts on lamin A/C.

NCBI and Uniprot Product Information

• NCBI GI #: 18379366
• NCBI GeneID: 10269
• NCBI Accession #: NP_005848
• NCBI GenBank Nucleotide #: NM_005857.4
• UniProt Accession #: O75844; Q8NDZ8; Q9UBQ2; B3KQI7; D3DPU7
• Molecular Weight: 54,813 Da
• NCBI Official Full Name: CAAX prenyl protease 1 homolog
• NCBI Official Synonym Full Names: zinc metallopeptidase STE24
• NCBI Official Symbol: ZMPSTE24
• NCBI Official Synonym Symbols: HGPS; PRO1; FACE1; STE24; FACE-1; Ste24p
• NCBI Protein Information: CAAX prenyl protease 1 homolog
• UniProt Protein Name: CAAX prenyl protease 1 homolog
• UniProt Gene Name: ZMPSTE24
• UniProt Synonym Gene Names: FACE1; STE24; FACE-1
• UniProt Entry Name: FACE1_HUMAN

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; EC 3.4.24.84; Membrane protein, integral

Chromosomal Location of Human Ortholog: 1p34

Cellular Component: membrane

Molecular Function: metalloexopeptidase activity

Biological Process: proteolysis

Disease: Mandibuloacral Dysplasia With Type B Lipodystrophy; Restrictive Dermopathy, Lethal