Mucolipin 1 Antibody - BSA Free

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

• Reactivity: Hu, Rt
• Applications: ICC/IF
• Clonality: Polyclonal
• Host: Rabbit
• Conjugate: Unconjugated
• Format: BSA Free

Mucolipin 1 Antibody - BSA Free Summary

• Description: Novus Biologicals Rabbit Mucolipin 1 Antibody - BSA Free (NBP3-17197) is a polyclonal antibody validated for use in ICC/IF. All Novus Biologicals antibodies are covered by our 100% guarantee.
• Immunogen: This antibody was developed against Recombinant Protein corresponding to amino acids: FLLGYSDGADDTFAAYTREQLYQAIFHAVDQYLALPDVSLGRYAYVRGGGDPWTNGSGLALCQRYYHRGHVDPANDTFDIDPMVVTDC
• Predicted Species: Rat (90%). Backed by our 100% Guarantee.
• Isotype: IgG
• Clonality: Polyclonal
• Host: Rabbit
• Gene: MCOLN1
• Purity: Affinity purified

Applications/Dilutions

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

Packaging, Storage & Formulations

• Storage: Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
• Buffer: PBS, pH 7.2, 40% glycerol
• Preservative: 0.02% Sodium Azide
• 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