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Key Documents

M8072

Sigma-Aldrich

Anti-Mucolipin-1 antibody, Mouse monoclonal

clone MLN128, purified from hybridoma cell culture

Sinonimo/i:

Anti-MCOLN1, Anti-ML4, Anti-MLIV, Anti-MST080, Anti-MSTP080, Anti-Mucolipidin, Anti-TRP-ML1, Anti-TRPM-L1, Anti-TRPML1, Anti-Transient receptor potential cation channel, mucolipin subfamily, member 1

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About This Item

Codice UNSPSC:
12352203
NACRES:
NA.41

Origine biologica

mouse

Coniugato

unconjugated

Forma dell’anticorpo

purified from hybridoma cell culture

Tipo di anticorpo

primary antibodies

Clone

MLN128, monoclonal

Forma fisica

buffered aqueous solution

PM

antigen ~110 kDa (additional bands may be observed)

Reattività contro le specie

human

Confezionamento

antibody small pack of 25 μL

Concentrazione

~2.0 mg/mL

tecniche

immunocytochemistry: suitable
indirect ELISA: suitable
western blot: 4-8 μg/mL using membrane fraction of HEK-293T expressing human mucolipin-1

N° accesso UniProt

Condizioni di spedizione

dry ice

Temperatura di conservazione

−20°C

modifica post-traduzionali bersaglio

unmodified

Informazioni sul gene

human ... MCOLN1(57192)

Descrizione generale

Monoclonal Anti-Mucolipin-1 (mouse IgG1 isotype) is derived from the hybridoma MLN128 produced by the fusion of mouse myeloma cells and splenocytes from BALB/c mice immunized with a recombinant fusion protein corresponding to amino acid 1.Mucolipin-1 is also termed TRP-ML1, MLN1, ML1 mucolipidin. MLN1 shares significant sequence homology with the TRP superfamily of cation channels.
Mucolipin-1 (MCOLN1) is a member of transient receptor potential (TRP) protein family. It is a cation channel present on endosomes and lysosomes.

Applicazioni

Monoclonal Anti-Mucolipin-1 has been used in:
  • enzyme linked immunosorbent assay (ELISA)
  • immunoblotting
  • immunocytochemistry.

Azioni biochim/fisiol

Mucolipin-1 (MCOLN1) is involved in the regulation of lysosomal trafficking. It aids in the transport of Ca2+ into the cytosol from the lumen, in response to the changing levels of phosphatidylinositol-3, 5-bisphosphate. Mutations in the gene encoding MCOLN1 have been shown to be associated with mucolipidosis type IV.
Mutations in the MCOLN1 gene is implicated in Mucolipidosis type IV (MLIV) is an autosomal recessive, neurodegenerative disorder. Rather, MLIV pathophysiology has been linked to deficiency in membrane trafficking, and organelle dynamics in the late endocytic pathway. Specifically, MLIV cells have been shown to accumulate autophagosomes, due to increased de novo autophagosome formation and due to delayed fusion of autophagosomes with late endosomes/lysosomes.

Stato fisico

Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.

Esclusione di responsabilità

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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Codice della classe di stoccaggio

10 - Combustible liquids

Classe di pericolosità dell'acqua (WGK)

WGK 1

Punto d’infiammabilità (°F)

Not applicable

Punto d’infiammabilità (°C)

Not applicable

Dispositivi di protezione individuale

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)


Certificati d'analisi (COA)

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Autophagic dysfunction in mucolipidosis type IV patients
Vergarajauregui S, et al.
Human Molecular Genetics, 17(17), 2723-2737 (2008)
Posttranslational cleavage and adaptor protein complex-dependent trafficking of mucolipin-1
Miedel MT, et al.
The Journal of Biological Chemistry, 281(18), 12751-12759 (2006)
Zhenxing Liu et al.
Biochemistry and cell biology = Biochimie et biologie cellulaire, 92(4), 279-286 (2014-06-25)
Lysosomotropic amines cause serious side effects such as cytoplasmic vacuolation and cell death. TRPML1 (also known as mucolipin1), a member of the transient receptor potential (TRP) protein family, may regulate fusion/fission of vesicles along the endocytic pathway and some aspects
Helen Waller-Evans et al.
Biochemical Society transactions, 43(3), 442-446 (2015-05-27)
TRPML1 is a ubiquitously expressed cation channel found on lysosomes and late endosomes. Mutations in TRPML1 cause mucolipidosis type IV and it has been implicated in Alzheimer's disease and HIV. However, the mechanisms by which TRPML1 activity is regulated are not
Wuyang Wang et al.
Proceedings of the National Academy of Sciences of the United States of America, 112(11), E1373-E1381 (2015-03-04)
Upon nutrient starvation, autophagy digests unwanted cellular components to generate catabolites that are required for housekeeping biosynthesis processes. A complete execution of autophagy demands an enhancement in lysosome function and biogenesis to match the increase in autophagosome formation. Here, we

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