06-1008 | Anti-VMA21 Antibody

06-1008
100 µg  
Retrieving price...
Price could not be retrieved
Minimum Quantity needs to be mulitiple of
Upon Order Completion More Information
You Saved ()
 
Request Pricing
Limited AvailabilityLimited Availability
In Stock 
Discontinued
Limited Quantities Available
Availability to be confirmed
    Remaining : Will advise
      Remaining : Will advise
      Will advise
      Contact Customer Service

      Special Offers

       

      Contact Customer Service

      Click To Print This Page

      Overview

      Replacement Information

      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H, R, M, Rhesus Macaque, ChpWB, ICC, IHCRbAffinity PurifiedPolyclonal Antibody
      Description
      Catalogue Number06-1008
      DescriptionAnti-VMA21 Antibody
      Alternate Names
      • VMA21 vacuolar H+-ATPase homolog (S. cerevisiae)
      • Myopathy with excessive autophagy protein
      • vacuolar ATPase assembly integral membrane protein VMA21
      • myopathy with excessive autophagy1
      • mNET4
      Background InformationVMA21, also known as NET4, is a necessary assembly chaperone for the V-ATPase proton pump of the cellular membrane. Deficiency of VMA21 is thought to be associated with XMEA (X-linked myopathy with excessive autophagy) which causes progressive vacuolation and skeletal muscle atrophy.
      References
      Product Information
      FormatAffinity Purified
      Control
      • Human fetal skeletal muscle tissue lysate.
      Presentation0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
      Applications
      ApplicationDetect VMA21 using this Anti-VMA21 Antibody validated for use in WB, IC, IH.
      Key Applications
      • Western Blotting
      • Immunocytochemistry
      • Immunohistochemistry
      Application NotesImmunohistochemistry Analysis: A representative lot was used by an independent laboratory in IH. (Wilkie, G.S., et al. (2011). Mol Cell Proteomics. 10(1)).

      Immunocytochemistry Analysis: A representative lot was used by an independent laboratory in IC. (Wilkie, G.S., et al. (2011). Mol Cell Proteomics. 10(1).
      Biological Information
      ImmunogenKLH-conjugated linear peptide corresponding to the N-terminus of human VMA21.
      EpitopeN-terminus
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      HostRabbit
      SpecificityThis antibody recognizes the N-terminus of VMA21.
      Species Reactivity
      • Human
      • Rat
      • Mouse
      • Rhesus Macaque
      • Chimpanzee
      Species Reactivity NoteDemonstrated to react with Human, Rat, and Mouse. Predicted to react with Rhesus Macaque, Orangutan, and Chimpanzee based on 100% sequence homology.
      Antibody TypePolyclonal Antibody
      Entrez Gene Number
      Entrez Gene SummarySummary: This gene encodes a vacuolar ATPase assembly integral membrane protein. Mutations of this gene result in an X-linked vacuolar myopathy with excessive autophagy.
      Gene Symbol
      • XMEA
      • MEAX
      Purification MethodAffinity Purfied
      UniProt Number
      UniProt SummaryFUNCTION: Required for the assembly of the V0 complex of the vacuolar ATPase (V-ATPase) in the endoplasmic reticulum.

      SUBUNIT STRUCTURE: Associates with the V0 complex of the vacuolar ATPase (V-ATPase).

      SUBCELLULAR LOCATION: Endoplasmic reticulum membrane; Multi-pass membrane protein. Endoplasmic reticulum-Golgi intermediate compartment membrane; Multi-pass membrane protein. Cytoplasmic vesicle › COPII-coated vesicle membrane; Multi-pass membrane protein.

      INVOLVEMENT IN DISEASE: Defects in VMA21 are the cause of X-linked myopathy with excessive autophagy (MEAX) [MIM:310440]. MEAX is a childhood-onset disease characterized by progressive vacuolation and atrophy of skeletal muscle. It is inherited in recessive fashion, affecting boys and sparing carrier females. Onset is in childhood, and patients exhibit weakness of the proximal muscles of the lower extremities, progressing slowly to involve other skeletal muscle groups over time. Other organs including the heart and brain are clinically unaffected. Phenotype is due to an increase of lysosomal pH from 4.7 to 5.2, which reduces lysosomal degradative ability and blocks autophagy. This reduces cellular free amino acids, which upregulates the mTOR pathway and mTOR-dependent macroautophagy, resulting in proliferation of large and ineffective autolysosomes that engulf sections of cytoplasm, merge together, and vacuolate the cell.

      SEQUENCE SIMILARITIES: Belongs to the VMA21 family.

      CAUTION: Protein characterization data are from. Due to a number of errors in the figure panels, the article has been retracted but the authors stand by the validity of the main results and conclusions.
      Molecular Weight~14 kDa observed
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blot in human fetal skeletal muscle tissue lysate.

      Western Blot Analysis: 1 µg/mL of this antibody detected VMA21 on 10 µg of human fetal skeletal muscle tissue lysate.
      Usage Statement
      • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
      Storage and Shipping Information
      Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
      Packaging Information
      Material Size100 µg
      Transport Information
      Supplemental Information
      Specifications

      Documentation

      SDS

      Title

      Safety Data Sheet (SDS) 

      Certificates of Analysis

      TitleLot Number
      Anti-VMA21 - 2455871 2455871
      Anti-VMA21 - 2562385 2562385
      Anti-VMA21 - NRG1901295 NRG1901295
      Anti-VMA21 -2831757 2831757

      References

      Reference overviewApplicationPub Med ID
      Several novel nuclear envelope transmembrane proteins identified in skeletal muscle have cytoskeletal associations.
      Wilkie, GS; Korfali, N; Swanson, SK; Malik, P; Srsen, V; Batrakou, DG; de las Heras, J; Zuleger, N; Kerr, AR; Florens, L; Schirmer, EC
      Molecular & cellular proteomics : MCP  10  M110.003129  2011

      Show Abstract Full Text Article
      Western Blotting20876400 20876400

      Brochure

      Title
      New Products: Volume 3, 2012