Rabbit anti-Human ZMPSTE24 Polyclonal Antibody | anti-ZMPSTE24 antibody

ZMPSTE24, CT (ZMPSTE24, FACE1, STE24, CAAX prenyl protease 1 homolog, Farnesylated proteins-converting enzyme 1, Prenyl protein-specific endoprotease 1, Zinc metalloproteinase Ste24 homolog) (FITC)

Cat. Num. AAA6365428

• Gene Names: ZMPSTE24; HGPS; PRO1; FACE1; STE24; FACE-1; Ste24p
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: ZMPSTE24; Polyclonal Antibody; CT (ZMPSTE24; FACE1; STE24; CAAX prenyl protease 1 homolog; Farnesylated proteins-converting enzyme 1; Prenyl protein-specific endoprotease 1; Zinc metalloproteinase Ste24 homolog) (FITC); Zinc metalloproteinase Ste24 homolog; anti-ZMPSTE24 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).

• Applicable Applications for anti-ZMPSTE24 antibody: Western Blot (WB), FLISA
• Application Notes: WB: 1:100-500; FLISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: ZMPSTE24 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 404-433 amino acids from the C-terminal region of human ZMPSTE24.
• Conjugate: FITC
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(ZMPSTE24 Antibody (C-term) western blot analysis in HepG2 cell line lysates (35ug/lane).This demonstrates the ZMPSTE24 antibody detected the ZMPSTE24 protein (arrow).)

Related Product Information for anti-ZMPSTE24 antibody

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.

Product Categories/Family for anti-ZMPSTE24 antibody

Antibodies; Matrix Metalloproteinases

References

Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010). Miner, J.H. Am. J. Med. Genet. A 152A (8), 2140-2141 (2010): Smigiel, R., et al. Am. J. Med. Genet. A 152A (2), 447-452 (2010): Fontaine-Bisson, B., et al. J. Mol. Med. 88(2):193-201(2010). Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009).

NCBI and Uniprot Product Information

• NCBI GI #: 18379366
• NCBI GeneID: 10269
• NCBI Accession #: NP_005848.2
• 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, 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