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anti-MCOLN1 antibody :: Rabbit MCOLN1 Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS9205163
Unit / Price
0.08 mL  /  $150 +1 FREE 8GB USB
0.4 mL  /  $340 +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   

MCOLN1, Polyclonal Antibody

★Popular Item★
 Also Known As   

MCOLN1 Antibody (C-term)

 Product Synonym Names    Mucolipin-1; MG-2; Mucolipidin; MCOLN1; ML4
 Product Gene Name   

anti-MCOLN1 antibody

[Similar Products]
 Antibody/Peptide Pairs    MCOLN1 peptide (MBS9219999) is used for blocking the activity of MCOLN1 antibody (MBS9205163)
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Immunogen Sequence Positions    531-560
 OMIM    252650
 3D Structure    ModBase 3D Structure for Q9GZU1
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 Clonality    Polyclonal
 Isotype    Rabbit Ig
 Clone Number    RB33839
 Host    Rabbit
 Species Reactivity    Human (Predicted Reactivity: Monkey, Mouse)
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 Specificity    This MCOLN1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 531-560 amino acids from the C-terminal region of human MCOLN1.
 Purity/Purification    Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)
 Form/Format    Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
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 Function    Cation channel probably playing a role in the endocytic pathway and in the control of membrane trafficking of proteins and lipids.Could play a major role in Ca(2+) transport regulating lysosomal exocytosis.
 Cellular Location    Cell membrane; Multi-pass membrane protein. Late endosome membrane; Multi-pass membrane protein. Lysosome membrane; Multi-pass membrane protein.
 Tissue Location    Widely expressed in adult and fetal tissues.
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 Preparation and Storage    Maintain refrigerated at 2-8 degree C for up to 2 weeks. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.
 Other Notes    Small volumes of anti-MCOLN1 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-MCOLN1 antibody

   This gene encodes a memberof the transient receptor
potential (TRP) cation channel gene family. The transmembrane
protein localizes to intracellular vesicular membranes including
lysosomes, and functions in the late endocytic pathway and in the
regulation of lysosomal exocytosis. The channel is permeable to
Ca(2+), Fe(2+), Na(+), K(+), and H(+), and is modulated by changes
in Ca(2+) concentration. Mutations in this gene result in
mucolipidosis type IV.
 Product Categories/Family for anti-MCOLN1 antibody    Signal Transduction
 Applications Tested/Suitable for anti-MCOLN1 antibody   

WB, IHC-P, ELISA

 Application Notes for anti-MCOLN1 antibody    WB~~1:1000
IHC-P~~1:10~50
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 Western Blot (WB) of anti-MCOLN1 antibody    MCOLN1 Antibody (C-term) western blot analysis in Jurkat cell line lysates (35ug/lane).This demonstrates the MCOLN1 antibody detected the MCOLN1 protein (arrow).
anti-MCOLN1 antibody Western Blot (WB) (WB) image
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 Immunohistochemistry (IHC) of anti-MCOLN1 antibody    MCOLN1 Antibody (C-term) (MBS9205163)immunohistochemistry analysis in formalin fixed and paraffin embedded human kidney tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of MCOLN1 Antibody (C-term) for immunohistochemistry. Clinical relevance has not been evaluated.
anti-MCOLN1 antibody Immunohistochemistry (IHC) (IHC) image
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NCBI/Uniprot data below describe general gene information for MCOLN1. It may not necessarily be applicable to this product.
 NCBI GI #    10092597
 NCBI GeneID    57192
 NCBI Accession #    NP_065394.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_020533.2 [Other Products]
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 UniProt Primary Accession #    Q9GZU1 [Other Products]
 UniProt Secondary Accession #    Q7Z4F7; Q9H292; Q9H4B3; Q9H4B5; D6W647 [Other Products]
 UniProt Related Accession #    Q9GZU1 [Other Products]
 Molecular Weight    65022
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 NCBI Official Full Name    mucolipin-1
 NCBI Official Synonym Full Names    mucolipin 1
 NCBI Official Symbol    MCOLN1 [Similar Products]
 NCBI Official Synonym Symbols   
ML4; MG-2; MLIV; MST080; TRPML1; MSTP080; TRP-ML1; TRPM-L1
[Similar Products]
 NCBI Protein Information    mucolipin-1
 UniProt Protein Name    Mucolipin-1
 UniProt Synonym Protein Names   
MG-2; Mucolipidin
 Protein Family    Mucolipin
 UniProt Gene Name    MCOLN1 [Similar Products]
 UniProt Synonym Gene Names    ML4 [Similar Products]
 UniProt Entry Name    MCLN1_HUMAN
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 NCBI Summary for MCOLN1    This gene encodes a memberof the transient receptor potential (TRP) cation channel gene family. The transmembrane protein localizes to intracellular vesicular membranes including lysosomes, and functions in the late endocytic pathway and in the regulation of lysosomal exocytosis. The channel is permeable to Ca(2+), Fe(2+), Na(+), K(+), and H(+), and is modulated by changes in Ca(2+) concentration. Mutations in this gene result in mucolipidosis type IV. [provided by RefSeq, Oct 2009]
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 UniProt Comments for MCOLN1    mucolipin 1: Cation channel probably playing a role in the endocytic pathway and in the control of membrane trafficking of proteins and lipids. Could play a major role in Ca(2+) transport regulating lysosomal exocytosis. Defects in MCOLN1 are the cause of mucolipidosis type IV (MLIV); also known as sialolipidosis. MLIV is an autosomal recessive lysosomal storage disorder characterized by severe psychomotor retardation and ophthalmologic abnormalities, including corneal opacity, retinal degeneration and strabismus. Storage bodies of lipids and water-soluble substances are seen by electron microscopy in almost every cell type of the patients. Most patients are unable to speak or walk independently and reach a maximal developmental level of 1-2 years. All patients have constitutive achlorhydia associated with a secondary elevation of serum gastrin levels. MLIV may be due to a defect in sorting and/or transport along the late endocytic pathway. MLIV is found at relatively high frequency among Ashkenazi Jews. Belongs to the transient receptor (TC 1.A.4) family. Polycystin subfamily. MCOLN1 sub-subfamily.

Protein type: Channel, cation; Membrane protein, integral; Membrane protein, multi-pass; Transporter, ion channel

Chromosomal Location of Human Ortholog: 19p13.2

Cellular Component: late endosome membrane; lysosomal membrane; integral to plasma membrane; cytoplasm; integral to membrane; plasma membrane; endosome membrane; receptor complex

Molecular Function: cation channel activity

Biological Process: cellular iron ion homeostasis; release of sequestered calcium ion into cytosol; transferrin transport; transmembrane transport; cation transport

Disease: Mucolipidosis Iv
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Product References and Citations for anti-MCOLN1 antibody

   Eichelsdoerfer, J.L., et al. J. Biol. Chem. 285(45):34304-34308(2010)
Curcio-Morelli, C., et al. J. Cell. Physiol. 222(2):328-335(2010)
Vergarajauregui, S., et al. J. Biol. Chem. 284(52):36357-36366(2009)
Ballif, B.A., et al. Mol. Cell Proteomics 3(11):1093-1101(2004)
LaPlante, J.M., et al. FEBS Lett. 532 (1-2), 183-187 (2002) :
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 Research Articles on MCOLN1    1. lysosomal adaptation to environmental cues such as nutrient levels requires mTOR/TFEB-dependent, lysosome-to-nucleus regulation of lysosomal ML1 channels and Ca(2+) signaling.
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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 anti-MCOLN1 antibodyPathways associated with anti-MCOLN1 antibody
 Reference Product  PubMed Publications
 PKD2 antibody  >2 publications with MCOLN1 and PKD2
 PKD2L1 antibody  >1 publications with MCOLN1 and PKD2L1
 TRPM1 antibody  >1 publications with MCOLN1 and TRPM1
 PDCD6 antibody  >1 publications with MCOLN1 and PDCD6
 Products by Pathway  Pathway Diagram
 Styrene Degradation Pathway antibodies  Styrene Degradation Pathway Diagram
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Diseases associated with anti-MCOLN1 antibodyOrgans/Tissues associated with anti-MCOLN1 antibody
 Disease Name  Pubmed Publications
 Mucolipidoses Antibodies  >67 publications with MCOLN1 and Mucolipidoses
 Hypertension Antibodies  >4 publications with MCOLN1 and Hypertension
 Atrophy Antibodies  >2 publications with MCOLN1 and Atrophy
 Kidney Diseases Antibodies  >2 publications with MCOLN1 and Kidney Diseases
 Growth Disorders Antibodies  >1 publications with MCOLN1 and Growth Disorders
 Cognition Disorders Antibodies  >1 publications with MCOLN1 and Cognition Disorders
 Hypertrophy Antibodies  >1 publications with MCOLN1 and Hypertrophy
 Inflammation Antibodies  >1 publications with MCOLN1 and Inflammation
 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >17 publications with MCOLN1 and Brain
 Blood Antibodies  >14 publications with MCOLN1 and Blood
 Eye Antibodies  >9 publications with MCOLN1 and Eye
 Kidney Antibodies  >7 publications with MCOLN1 and Kidney
 Lung Antibodies  >7 publications with MCOLN1 and Lung
 Heart Antibodies  >6 publications with MCOLN1 and Heart
 Skin Antibodies  >6 publications with MCOLN1 and Skin
 Nerve Antibodies  >4 publications with MCOLN1 and Nerve
 Stomach Antibodies  >3 publications with MCOLN1 and Stomach
 Vascular Antibodies  >3 publications with MCOLN1 and Vascular
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