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Mucolipin 1 Antibody

Novus Biologicals, part of Bio-Techne | Catalog # NBP3-17197

Novus Biologicals, part of Bio-Techne
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NBP3-17197-100ul
NBP3-17197-25ul

Key Product Details

Species Reactivity

Validated:

Human

Predicted:

Rat (90%). Backed by our 100% Guarantee.

Applications

Immunocytochemistry/ Immunofluorescence

Label

Unconjugated

Antibody Source

Polyclonal Rabbit IgG

Concentration

Concentrations vary lot to lot. See vial label for concentration. If unlisted please contact technical services.

Product Specifications

Immunogen

This antibody was developed against Recombinant Protein corresponding to amino acids: FLLGYSDGADDTFAAYTREQLYQAIFHAVDQYLALPDVSLGRYAYVRGGGDPWTNGSGLALCQRYYHRGHVDPANDTFDIDPMVVTDC

Clonality

Polyclonal

Host

Rabbit

Isotype

IgG

Scientific Data Images for Mucolipin 1 Antibody

Immunocytochemistry/ Immunofluorescence: Mucolipin 1 Antibody [NBP3-17197]

Immunocytochemistry/ Immunofluorescence: Mucolipin 1 Antibody [NBP3-17197]

Immunocytochemistry/Immunofluorescence: Mucolipin 1 Antibody [NBP3-17197] - Staining of human cell line U-2 OS shows localization to nucleoplasm, the Golgi apparatus & vesicles.

Applications for Mucolipin 1 Antibody

Application
Recommended Usage

Immunocytochemistry/ Immunofluorescence

0.25-2 ug/ml
Application Notes
Immunocytochemistry/Immunofluorescence, PFA/Triton X-100 is recommended for fixation/permeabilization.

Formulation, Preparation, and Storage

Purification

Affinity purified

Formulation

PBS, pH 7.2, 40% glycerol

Preservative

0.02% Sodium Azide

Concentration

Concentrations vary lot to lot. See vial label for concentration. If unlisted please contact technical services.

Shipping

The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.

Stability & Storage

Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.

Background: Mucolipin 1

Mucolipin-1 (MCOLN1) is a 65kDa member of the TRPML channel family, which is a subgroup of the large protein family of TRP ion channels. Mucolipin 1 is thought to channel iron ions across the endosome/lysosome membrane into the cell, so defective MCOLN1 may cause cellular iron deficiency.

Defects in MCOLN1 are known to cause 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.

Long Name

Mucolipin-1

Alternate Names

MCOLN1, MG-2, ML1, ML4, MSTP080, Mucolipidin, TRPML1

Gene Symbol

MCOLN1

Additional Mucolipin 1 Products

Product Documents for Mucolipin 1 Antibody

Certificate of Analysis

To download a Certificate of Analysis, please enter a lot number in the search box below.

Product Specific Notices for Mucolipin 1 Antibody

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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