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ZMPSTE24 peptide :: FACE1 / ZMPSTE24 Peptide

Scan QR to view Datasheet Catalog #    MBS426490 ZMPSTE24 peptide
Unit / Price
0.1 mg  /  $190 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

FACE1 / ZMPSTE24, Peptide

 Also Known As   

FACE1 / ZMPSTE24 Immunizing Peptide

 Product Synonym Names    FACE1; ZMPSTE24; zinc metallopeptidase (STE24 homolog, yeast); FLJ14968; MADB; STE24; Ste24p; CAAX prenyl protease; farnesylated-proteins converting enzyme 1; prenyl protein-specific endoprotease 1; zinc metalloproteinase (STE24 homolog, yeast); zinc met; FACE1 / ZMPSTE24
 Product Gene Name   

ZMPSTE24 peptide

[Similar Products]
 Product Synonym Gene Name    FACE1 [Similar Products]
 Antibody/Peptide Pairs    FACE1 / ZMPSTE24 peptide (MBS426490) is used for blocking the activity of FACE1/ZMPSTE24 antibody (MBS420669)
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Sequence    C-ERLQALKT MKQH
 OMIM    275210
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 Species Reactivity    Human
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 Form/Format    100ug of dried peptide
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 Preparation and Storage    Shipped at ambient temperature, store at -20 degree C
 ISO Certification    Manufactured in an ISO 9001:2015 Certified Laboratory.
 Other Notes    Small volumes of ZMPSTE24 peptide 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.
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NCBI/Uniprot data below describe general gene information for ZMPSTE24. It may not necessarily be applicable to this product.
 NCBI GI #    18379366
 NCBI GeneID    10269
 NCBI Accession #    NP_005848.2 [Other Products]
 NCBI GenBank Nucleotide #    NM_005857.4 [Other Products]
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 UniProt Secondary Accession #    Q8NDZ8; Q9UBQ2; B3KQI7; D3DPU7 [Other Products]
 UniProt Related Accession #    O75844 [Other Products]
 Molecular Weight    54,813 Da
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 NCBI Official Full Name    CAAX prenyl protease 1 homolog
 NCBI Official Synonym Full Names    zinc metallopeptidase STE24
 NCBI Official Symbol    ZMPSTE24 [Similar Products]
 NCBI Official Synonym Symbols   
HGPS; PRO1; FACE1; STE24; FACE-1; Ste24p
[Similar Products]
 NCBI Protein Information    CAAX prenyl protease 1 homolog
 UniProt Protein Name    CAAX prenyl protease 1 homolog
 UniProt Synonym Protein Names   
Farnesylated proteins-converting enzyme 1; FACE-1; Prenyl protein-specific endoprotease 1; Zinc metalloproteinase Ste24 homolog
 UniProt Gene Name    ZMPSTE24 [Similar Products]
 UniProt Synonym Gene Names    FACE1; STE24; FACE-1 [Similar Products]
 UniProt Entry Name    FACE1_HUMAN
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 NCBI Summary for ZMPSTE24    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]
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 UniProt Comments for ZMPSTE24    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: Protease; EC 3.4.24.84; Membrane protein, multi-pass; Membrane protein, integral

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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 Research Articles on ZMPSTE24    1. complete loss-of-function of ZMPSTE24 leads to RD, whereas other less severe phenotypes are associated with at least one haploinsufficient allele.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with ZMPSTE24 peptidePathways associated with ZMPSTE24 peptide
 Reference Product  PubMed Publications
 LMNA peptide  >79 publications with ZMPSTE24 and LMNA
 RCE1 peptide  >9 publications with ZMPSTE24 and RCE1
 ICMT peptide  >7 publications with ZMPSTE24 and ICMT
 FNTB peptide  >2 publications with ZMPSTE24 and FNTB
 Products by Pathway  Pathway Diagram
 Adipogenesis Pathway antibodies  Adipogenesis Pathway Diagram
 Terpenoid Backbone Biosynthesis Pathway antibodies  Terpenoid Backbone Biosynthesis Pathway Diagram
 Terpenoid Backbone Biosynthesis Pathway antibodies  Terpenoid Backbone Biosynthesis Pathway Diagram
Diseases associated with ZMPSTE24 peptideOrgans/Tissues associated with ZMPSTE24 peptide
 Disease Name  Pubmed Publications
 Progeria Antibodies  >96 publications with ZMPSTE24 and Progeria
 Lipodystrophy Antibodies  >38 publications with ZMPSTE24 and Lipodystrophy
 Tight skin contracture syndrome, lethal Antibodies  >8 publications with ZMPSTE24 and Tight skin contracture syndrome, lethal
 Craniofacial Abnormalities Antibodies  >7 publications with ZMPSTE24 and Craniofacial Abnormalities
 Mandibular Diseases Antibodies  >6 publications with ZMPSTE24 and Mandibular Diseases
 Acro-Osteolysis Antibodies  >6 publications with ZMPSTE24 and Acro-Osteolysis
 Weight Loss Antibodies  >6 publications with ZMPSTE24 and Weight Loss
 Heart Diseases Antibodies  >5 publications with ZMPSTE24 and Heart Diseases
 HIV-Associated Lipodystrophy Syndrome Antibodies  >5 publications with ZMPSTE24 and HIV-Associated Lipodystrophy Syndrome
 Fetal Growth Retardation Antibodies  >4 publications with ZMPSTE24 and Fetal Growth Retardation
 Organ/Tissue Name  Pubmed Publications
 Skin Antibodies  >52 publications with ZMPSTE24 and Skin
 Bone Antibodies  >32 publications with ZMPSTE24 and Bone
 Muscle Antibodies  >16 publications with ZMPSTE24 and Muscle
 Connective Tissue Antibodies  >15 publications with ZMPSTE24 and Connective Tissue
 Blood Antibodies  >14 publications with ZMPSTE24 and Blood
 Adipose Tissue Antibodies  >9 publications with ZMPSTE24 and Adipose Tissue
 Mouth Antibodies  >8 publications with ZMPSTE24 and Mouth
 Liver Antibodies  >6 publications with ZMPSTE24 and Liver
 Brain Antibodies  >5 publications with ZMPSTE24 and Brain
 Vascular Antibodies  >4 publications with ZMPSTE24 and Vascular
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