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  • 产品名称:MCOLN1, Blocking Peptide

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简单介绍:
MCOLN1, Blocking Peptide
详情介绍:
Product Name

MCOLN1, Blocking Peptide

Full Product Name

MCOLN1 Antibody (C-term) Blocking peptide

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

MCOLN1 blocking peptide

[Similar Products]
Product Synonym Gene Name
ML4[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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OMIM
252650
3D Structure
ModBase 3D Structure for Q9GZU1
Specificity
The synthetic peptide sequence used to generate the antibody was selected from the C-term region of MCOLN1. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular *****.
Form/Format
Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
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 ***** and fetal tissues.
Preparation and Storage
Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.
Other Notes
Small volumes of MCOLN1 blocking 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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Related Product Information for
MCOLN1 blocking peptide
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.
NCBI/Uniprot data below describe general gene information for MCOLN1. It may not necessarily be applicable to this product.
NCBI GI #
50401163
NCBI GeneID
57192
NCBI Accession #
Q9GZU1.1 [Other Products]
UniProt Primary Accession #
Q9GZU1 [Other Products]
UniProt Secondary Accession #
Q7Z4F7; Q9H292; Q9H4B3; Q9H4B5; D6W647[Other Products]
UniProt Related Accession #
Q9GZU1[Other Products]
Molecular Weight
65,022 Da
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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: Transporter, ion channel; Membrane protein, integral; Channel, cation; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 19p13.2

Cellular Component: cytoplasm; endosome membrane; lysosomal membrane; plasma membrane; receptor complex

Molecular Function: calcium channel activity; iron ion transmembrane transporter activity

Biological Process: transferrin transport

Disease: Mucolipidosis Iv
Research Articles on MCOLN1
1. This review summarizes the current understanding of TRPML1 activation and regulation
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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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