PMID- 24904279
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20140624
LR  - 20211021
IS  - 1662-5102 (Print)
IS  - 1662-5102 (Electronic)
IS  - 1662-5102 (Linking)
VI  - 8
DP  - 2014
TI  - The sodium leak channel, NALCN, in health and disease.
PG  - 132
LID - 10.3389/fncel.2014.00132 [doi]
LID - 132
AB  - Ion channels are crucial components of cellular excitability and are involved in 
      many neurological diseases. This review focuses on the sodium leak, G 
      protein-coupled receptors (GPCRs)-activated NALCN channel that is predominantly 
      expressed in neurons where it regulates the resting membrane potential and 
      neuronal excitability. NALCN is part of a complex that includes not only GPCRs, 
      but also UNC-79, UNC-80, NLF-1 and src family of Tyrosine kinases (SFKs). There 
      is growing evidence that the NALCN channelosome critically regulates its ion 
      conduction. Both in mammals and invertebrates, animal models revealed an 
      involvement in many processes such as locomotor behaviors, sensitivity to 
      volatile anesthetics, and respiratory rhythms. There is also evidence that 
      alteration in this NALCN channelosome can cause a wide variety of diseases. 
      Indeed, mutations in the NALCN gene were identified in Infantile Neuroaxonal 
      Dystrophy (INAD) patients, as well as in patients with an Autosomal Recessive 
      Syndrome with severe hypotonia, speech impairment, and cognitive delay. Deletions 
      in NALCN gene were also reported in diseases such as 13q syndrome. In addition, 
      genes encoding NALCN, NLF- 1, UNC-79, and UNC-80 proteins may be susceptibility 
      loci for several diseases including bipolar disorder, schizophrenia, Alzheimer's 
      disease, autism, epilepsy, alcoholism, cardiac diseases and cancer. Although the 
      physiological role of the NALCN channelosome is poorly understood, its 
      involvement in human diseases should foster interest for drug development in the 
      near future. Toward this goal, we review here the current knowledge on the NALCN 
      channelosome in physiology and diseases.
FAU - Cochet-Bissuel, Maud
AU  - Cochet-Bissuel M
AD  - Institut de Genomique Fonctionnelle, CNRS UMR 5203, Universites Montpellier 1&2 
      Montpellier, France ; INSERM, U 661 Montpellier, France ; LabEx 'Ion Channel 
      Science and Therapeutics' Montpellier, France.
FAU - Lory, Philippe
AU  - Lory P
AD  - Institut de Genomique Fonctionnelle, CNRS UMR 5203, Universites Montpellier 1&2 
      Montpellier, France ; INSERM, U 661 Montpellier, France ; LabEx 'Ion Channel 
      Science and Therapeutics' Montpellier, France.
FAU - Monteil, Arnaud
AU  - Monteil A
AD  - Institut de Genomique Fonctionnelle, CNRS UMR 5203, Universites Montpellier 1&2 
      Montpellier, France ; INSERM, U 661 Montpellier, France ; LabEx 'Ion Channel 
      Science and Therapeutics' Montpellier, France.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20140520
PL  - Switzerland
TA  - Front Cell Neurosci
JT  - Frontiers in cellular neuroscience
JID - 101477935
PMC - PMC4033012
OTO - NOTNLM
OT  - NALCN
OT  - UNC-79
OT  - UNC-80
OT  - excitability
OT  - ion channel
EDAT- 2014/06/07 06:00
MHDA- 2014/06/07 06:01
PMCR- 2014/01/01
CRDT- 2014/06/07 06:00
PHST- 2014/03/05 00:00 [received]
PHST- 2014/04/28 00:00 [accepted]
PHST- 2014/06/07 06:00 [entrez]
PHST- 2014/06/07 06:00 [pubmed]
PHST- 2014/06/07 06:01 [medline]
PHST- 2014/01/01 00:00 [pmc-release]
AID - 10.3389/fncel.2014.00132 [doi]
PST - epublish
SO  - Front Cell Neurosci. 2014 May 20;8:132. doi: 10.3389/fncel.2014.00132. 
      eCollection 2014.