Mucolipin 1 Antibody

Novus Biologicals, part of Bio-Techne | Catalog # NBP2-87859

Novus Biologicals, part of Bio-Techne
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NBP2-87859
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Key Product Details

Species Reactivity

Human

Applications

Western Blot

Label

Unconjugated

Antibody Source

Polyclonal Rabbit

Concentration

0.5 mg/ml

View all Mucolipin 1 Antibodies »

Product Specifications

Immunogen

The immunogen is a synthetic peptide directed towards the N-terminal region of human Mucolipin 1. Peptide sequence: FRHLFLLGYSDGADDTFAAYTREQLYQAIFHAVDQYLALPDVSLGRYAYV The peptide sequence for this immunogen was taken from within the described region.

Clonality

Polyclonal

Host

Rabbit

Scientific Data Images for Mucolipin 1 Antibody

Western Blot: Mucolipin 1 Antibody [NBP2-87859]

Western Blot: Mucolipin 1 Antibody [NBP2-87859]

Western Blot: Mucolipin 1 Antibody [NBP2-87859] - Host: Rabbit. Target Name: MCOLN1. Sample Tissue: Human 786-0 Whole Cell. Antibody Dilution: 1ug/ml
Western Blot: Mucolipin 1 Antibody [NBP2-87859]

Western Blot: Mucolipin 1 Antibody [NBP2-87859]

Western Blot: Mucolipin 1 Antibody [NBP2-87859] - Host: Rabbit. Target Name: MCOLN1. Sample Tissue: Human Fetal Lung. Antibody Dilution: 1.0ug/ml
Western Blot: Mucolipin 1 Antibody [NBP2-87859]

Western Blot: Mucolipin 1 Antibody [NBP2-87859]

Western Blot: Mucolipin 1 Antibody [NBP2-87859] - Host: Rabbit. Target Name: MCOLN1. Sample Tissue: Human 786-0 Whole Cell. Antibody Dilution: 1ug/ml
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Applications for Mucolipin 1 Antibody

Application
Recommended Usage

Western Blot

1.0 ug/ml

Formulation, Preparation, and Storage

Purification

Affinity purified

Formulation

PBS, 2% Sucrose

Preservative

0.09% Sodium Azide

Concentration

0.5 mg/ml

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

Product Datasheets

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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