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ZMPSTE24, Polyclonal Antibody

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ZMPSTE24, Polyclonal Antibody


ZMPSTE24, Polyclonal Antibody  的詳細介紹
Product Name

ZMPSTE24, Polyclonal Antibody

Full Product Name

ZMPSTE24 (Zinc Metalloproteinase Ste24 Homolog, CAAX Prenyl Protease 1 Homolog, Farnesylated Proteins Converting Enzyme 1, FACE 1, FACE1, FACE-1, FLJ14968, MADB, Prenyl Protein Specific Endoprotease 1, STE24)

Product Synonym Names
Anti -ZMPSTE24 (Zinc Metalloproteinase Ste24 Homolog, CAAX Prenyl Protease 1 Homolog, Farnesylated Proteins Converting Enzyme 1, FACE 1, FACE1, FACE-1, FLJ14968, MADB, Prenyl Protein Specific Endoprotease 1, STE24)
Product Gene Name

anti-ZMPSTE24 antibody

[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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Chromosome Location
Chromosome: 1; NC_000001.10 (40723733..40759856). Location: 1p34
OMIM
275210
3D Structure
ModBase 3D Structure for O75844
Clonality
Polyclonal
Host
Rabbit
Species Reactivity
Human
Specificity
Recognizes human ZMPSTE24.
Purity/Purification
Affinity Purified
Purified by peptide immunoaffinity chromatography.
Form/Format
Supplied as a liquid in Tris-glycine, 150mM sodium chloride, pH 7.2.
Immunogen
A synthetic peptide made to an N-terminal portion of the human ZMPSTE24 protein sequence (between residues 1-100). [Swiss-Prot O75844]. Homology: 100% Mu, Ze, Xp
Cellular Localization
Integral membrane, primarily ER
Positive Control
Human or mouse testis lysate
Preparation and Storage
May be stored at 4 degree C for short-term only. For long-term storage and to avoid repeated freezing and thawing, add sterile glycerol (40-50%), aliquot and store at -20 degree C. Aliquots are stable for at least 3 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
Other Notes
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.
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Related Product Information for
anti-ZMPSTE24 antibody
ZMPSTE24 is a zinc metalloprotease involved in the post-translational processing of LMNA precursors. Mutations in ZMPSTE24 have been associated with human diseases, often due to the accumulation of LMNA precursors. It is possible that ZMPSTE24, due to its association with LMNA, plays some role in human ageing.
Product Categories/Family for anti-ZMPSTE24 antibody
Antibodies; Abs to Enzymes, Protease (Proteinase, Peptidase)
Applications Tested/Suitable for anti-ZMPSTE24 antibody
Western Blot (WB)
Application Notes for anti-ZMPSTE24 antibody
Suitable for use in Western Blot.
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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 #
119627639
NCBI GeneID
10269
UniProt Primary Accession #
O75844 [Other Products]
UniProt Secondary Accession #
Q8NDZ8; Q9UBQ2; B3KQI7; D3DPU7[Other Products]
UniProt Related Accession #
O75844[Other Products]
Molecular Weight
54,813 Da[Similar Products]
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NCBI Official Full Name
zinc metallopeptidase (STE24 homolog, yeast), isoform CRA_a
NCBI Official Synonym Full Names
zinc metallopeptidase (STE24 homolog, S. cerevisiae)
NCBI Official Symbol
ZMPSTE24??[Similar Products]
NCBI Official Synonym Symbols
HGPS; PRO1; FACE1; STE24; FACE-1; Ste24p; FLJ14968
??[Similar Products]
NCBI Protein Information
CAAX prenyl protease 1 homolog; OTTHUMP00000006394; Hutchinson-Gilford progeria syndrome; zinc metalloproteinase Ste24 homolog; prenyl protein-specific endoprotease 1; farnesylated proteins-converting enzyme 1; farnesylated-proteins converting enzyme 1
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??[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]
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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: EC 3.4.24.84; Membrane protein, integral; Protease; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 1p34

Cellular Component: endoplasmic reticulum membrane; membrane; integral to membrane; nuclear inner membrane

Molecular Function: metalloexopeptidase activity; metal ion binding; metalloendopeptidase activity

Biological Process: prenylated protein catabolic process; proteolysis; nuclear membrane organization and biogenesis

Disease: Restrictive Dermopathy, Lethal; Mandibuloacral Dysplasia With Type B Lipodystrophy
Research Articles on ZMPSTE24
1. In patients with mandibuloacral dysplasia due to ZMPSTE24 mutations, the onset of disease manifestations such as thin skin and micrognathia occurs as early as 5 months of age.
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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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