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Code
OSM00039W
$240USD    Buy Now 

ID Tag
Rb0064-180807-WS

Immunogen
A synthetic peptide from human Mucolipin 1 conjugated to blue carrier protein was used as the antigen.

Accession

Also known
Mucolipin-1, Mucolipidin, Mcoln1, Mucolipin 1, Mcoln1, MCLN1, TRPML1, MGC7172, mucolipidin, 2210015I05Rik, Mucolipin-1, MG-2, ML4, MSTP080, MLIV, TRP-ML1, TRPM-L1, MCOLN1 mucolipin 1

Target
FUNCTION: Cationic 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.
SUBUNIT: Forms multimeric complexes.
SUBCELLULAR LOCATION: Cell membrane; Multi-pass membrane protein. Late endosome membrane; Multi-pass membrane protein. Lysosome membrane; Multi-pass membrane protein.
TISSUE SPECIFICITY: Widely expressed in adult and fetal tissues.
DISEASE: 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.
MISCELLANEOUS: Channel function is transiently modulated by changes in Ca(2+), and inhibited by a reduction of pH; pH changes modify the aggregation state of unitary channels

Storage
Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.

Expiry Date
12 months after reconstitution

Shipping
This item will be shipped to you at ambient temperature in a lyophilised form.

References
1. Bassi M.T, et al. Am. J. Hum. Genet. 67:1110-1120(2000).

Limitation
For research use only

Code
OSM00039W
$240USD    Buy Now 

Unit size
100 µl

Conjugate
Unconjugated antibody

Host
NZ white rabbit

Purity
Whole serum

Clonality
Polyclonal

Isotype
Polyclonal, whole serum

Applications
IHC, WB. A dilution of 1: 1000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.

Specificity
Specific for Mucolipin 1, isoforms 1 and 2.

Spcs X-react.
Human. Other species not yet tested.

Format
Lyophilised

Reconstitution
Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.

Note
Control peptide is available at $120 per 50 μg. Please enquire sales@osenses.com

⚬ Products
Rabbit antibody to Mucolipin 1
Content

Download Protocols

APES coating of slides
SDS Loading Buffer
IHC-P on Autostainer
G1 Lysing Buffer
WB Protocol online
IHC-P HIER (Tris-EDTA, pH 9)
4% PFA
Davidson's fix (modified)
....Tissue lysate perparation
....G2 Lysing Buffer
....R Lysing Buffer
....G3 Lysing Buffer
....G4 Lysing Buffer
....G5 Lysing Buffer
.G6 Lysing Buffer
Content

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