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anti-MCOLN1 antibody :: Rabbit MCOLN1 Polyclonal Antibody

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Catalog # MBS9205163
Unit / Price
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  0.08 mL  /  $165 +1 FREE 8GB USB
  0.4 mL  /  $370 +1 FREE 8GB USB
Product Name

MCOLN1, Polyclonal Antibody

Popular Item
Full Product Name

MCOLN1 Antibody (C-term)

Product Synonym Names
Mucolipin-1; MG-2; Mucolipidin; MCOLN1; ML4
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.
Immunogen Sequence Positions
531-560
OMIM
252650
3D Structure
ModBase 3D Structure for Q9GZU1
Clonality
Polyclonal
Isotype
Rabbit Ig
Clone Number
RB33839
Host
Rabbit
Species Reactivity
Human (Predicted Reactivity: Monkey, Mouse)
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.
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.
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.
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
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

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
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
NCBI/Uniprot data below describe general gene information for MCOLN1. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
65022
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
UniProt Gene Name
MCOLN1  [Similar Products]
UniProt Synonym Gene Names
UniProt Entry Name
MCLN1_HUMAN
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]
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
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) :

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.

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