Mucolipin 1 Antibody - BSA Free

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] - Host: Rabbit. Target Name: MCOLN1. Sample Tissue: Human Fetal Lung. Antibody Dilution: 1.0ug/ml

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] - Host: Rabbit. Target: MCOLN1. Positive control (+): Human Placenta (PL). Negative control (-): Human liver (LI). Antibody concentration: 1ug/ml

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

• Reactivity: Hu
• Applications: WB
• Clonality: Polyclonal
• Host: Rabbit
• Conjugate: Unconjugated
• Format: BSA Free
• Concentration: 0.5 mg/ml

Mucolipin 1 Antibody - BSA Free Summary

• Description: Novus Biologicals Rabbit Mucolipin 1 Antibody - BSA Free (NBP2-87859) is a polyclonal antibody validated for use in WB. All Novus Biologicals antibodies are covered by our 100% guarantee.
• 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
• Gene: MCOLN1
• Purity: Affinity purified

Applications/Dilutions

• Dilutions:
  • Western Blot 1.0 ug/ml

Packaging, Storage & Formulations

• Storage: Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
• Buffer: PBS, 2% Sucrose
• Preservative: 0.09% Sodium Azide
• Concentration: 0.5 mg/ml
• Purity: Affinity purified

Alternate Names for Mucolipin 1 Antibody - BSA Free

• MCOLN1
• MG-2
• ML1
• ML4
• ML4TRP-ML1
• MLIV
• MST080
• MSTP080
• Mucolipidin
• mucolipidosis type IV protein
• Mucolipin 1
• mucolipin-1
• TRPML1
• TRP-ML1
• TRPM-L1

Background

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.

Limitations

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.

Bioinformatics

• Gene Symbol: MCOLN1