Target type: molecularfunction
Enables passage of a chloride ion through a transmembrane channel that opens when ATP is bound by the channel complex or one of its constituent parts on the intracellular side of the plasma membrane. [PMID:24727426, PMID:9922375]
Intracellularly ATP-gated chloride channel activity describes the function of a specific type of ion channel that is activated by intracellular ATP binding. These channels play a crucial role in regulating intracellular chloride ion concentration, which in turn influences cell volume, membrane potential, and various cellular processes. Here's a detailed breakdown of their molecular function:
**1. Channel Structure and Composition:**
- These channels are typically composed of multiple protein subunits that assemble to form a pore through the cell membrane.
- The specific structure and subunit composition can vary depending on the cell type and the specific channel involved.
**2. ATP Binding and Activation:**
- ATP binding to a specific site on the channel protein triggers a conformational change.
- This change opens the channel pore, allowing chloride ions to flow across the cell membrane.
**3. Chloride Ion Transport:**
- Chloride ions move through the channel down their electrochemical gradient.
- This movement can either be into or out of the cell, depending on the relative chloride ion concentrations inside and outside the cell.
**4. Regulation and Modulation:**
- The activity of intracellularly ATP-gated chloride channels can be regulated by various factors, including:
- Intracellular ATP concentration: Higher ATP levels increase channel activity.
- Other intracellular signaling molecules: Some messengers can modulate channel activity, either enhancing or inhibiting it.
- Membrane potential: Changes in membrane potential can influence channel opening and closing.
**5. Physiological Roles:**
- These channels play diverse roles in cellular function, including:
- Cell volume regulation: By controlling chloride ion movement, they help regulate cell volume and prevent swelling or shrinking.
- Membrane potential regulation: Chloride ion fluxes contribute to membrane potential changes, which are essential for neuronal signaling, muscle contraction, and other cellular processes.
- Cellular signaling: Chloride channels can participate in signaling pathways by regulating the intracellular chloride concentration, which influences the activity of other ion channels and signaling molecules.
- Cell survival and apoptosis: Some studies suggest that these channels may play a role in cell survival and apoptosis by influencing ion homeostasis and signaling pathways.
**6. Disease Relevance:**
- Dysregulation of intracellularly ATP-gated chloride channels has been implicated in various diseases, including:
- Cystic fibrosis: Mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR), a chloride channel, cause cystic fibrosis.
- Epilepsy: Abnormal chloride channel activity can contribute to seizures in epilepsy.
- Cancer: Some cancers exhibit altered chloride channel expression and activity, which can contribute to tumor growth and invasion.
In summary, intracellularly ATP-gated chloride channels are essential for maintaining cellular homeostasis, regulating membrane potential, and participating in various cellular signaling processes. Their dysfunction can contribute to a range of diseases, highlighting their importance in health and disease.'
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Protein | Definition | Taxonomy |
---|---|---|
Cystic fibrosis transmembrane conductance regulator | A cystic fibrosis transmembrane conductance regulator that is encoded in the genome of human. [PRO:CNA, UniProtKB:P13569] | Homo sapiens (human) |
Compound | Definition | Classes | Roles |
---|---|---|---|
glyburide | glyburide : An N-sulfonylurea that is acetohexamide in which the acetyl group is replaced by a 2-(5-chloro-2-methoxybenzamido)ethyl group. Glyburide: An antidiabetic sulfonylurea derivative with actions like those of chlorpropamide | monochlorobenzenes; N-sulfonylurea | anti-arrhythmia drug; EC 2.7.1.33 (pantothenate kinase) inhibitor; EC 3.6.3.49 (channel-conductance-controlling ATPase) inhibitor; hypoglycemic agent |
methyl anthranilate | methyl anthranilate : A benzoate ester that is the methyl ester of anthranilic acid. | benzoate ester | flavouring agent; metabolite |
telmisartan | telmisartan : A member of the class of benzimidazoles used widely in the treatment of hypertension. Telmisartan: A biphenyl compound and benzimidazole derivative that acts as an angiotensin II type 1 receptor antagonist. It is used in the management of HYPERTENSION. | benzimidazoles; biphenyls; carboxybiphenyl | angiotensin receptor antagonist; antihypertensive agent; EC 3.4.15.1 (peptidyl-dipeptidase A) inhibitor; environmental contaminant; xenobiotic |
tadalafil | benzodioxoles; pyrazinopyridoindole | EC 3.1.4.35 (3',5'-cyclic-GMP phosphodiesterase) inhibitor; vasodilator agent | |
7-n-butyl-6-(4'-hydroxyphenyl)-5h-pyrrolo(2,3b)pyrazine | |||
3-((3-trifluoromethyl)phenyl)-5-((3-carboxyphenyl)methylene)-2-thioxo-4-thiazolidinone | 3-((3-trifluoromethyl)phenyl)-5-((3-carboxyphenyl)methylene)-2-thioxo-4-thiazolidinone: a cystic fibrosis transmembrane conductance regulator inhibitor; structure in first source | ||
uccf-029 | organic heterotricyclic compound; organooxygen compound | ||
quercetin | 7-hydroxyflavonol; pentahydroxyflavone | antibacterial agent; antineoplastic agent; antioxidant; Aurora kinase inhibitor; chelator; EC 1.10.99.2 [ribosyldihydronicotinamide dehydrogenase (quinone)] inhibitor; geroprotector; phytoestrogen; plant metabolite; protein kinase inhibitor; radical scavenger | |
apigenin | Chamomile: Common name for several daisy-like plants (MATRICARIA; TRIPLEUROSPERMUM; ANTHEMIS; CHAMAEMELUM) native to Europe and Western Asia, now naturalized in the United States and Australia. | trihydroxyflavone | antineoplastic agent; metabolite |
rutin | Hydroxyethylrutoside: Monohydroxyethyl derivative of rutin. Peripheral circulation stimulant used in treatment of venous disorders. | disaccharide derivative; quercetin O-glucoside; rutinoside; tetrahydroxyflavone | antioxidant; metabolite |
genistein | 7-hydroxyisoflavones | antineoplastic agent; EC 5.99.1.3 [DNA topoisomerase (ATP-hydrolysing)] inhibitor; geroprotector; human urinary metabolite; phytoestrogen; plant metabolite; tyrosine kinase inhibitor | |
vx-770 | ivacaftor : An aromatic amide obtained by formal condensation of the carboxy group of 4-oxo-1,4-dihydroquinoline-3-carboxylic acid with the amino group of 5-amino-2,4-di-tert-butylphenol. Used for the treatment of cystic fibrosis. ivacaftor: a CFTR potentiator; structure in first source | aromatic amide; monocarboxylic acid amide; phenols; quinolone | CFTR potentiator; orphan drug |
lumacaftor | lumacaftor : An aromatic amide obtained by formal condensation of the carboxy group of 1-(2,2-difluoro-1,3-benzodioxol-5-yl)cyclopropane-1-carboxylic acid with the aromatic amino group of 3-(6-amino-3-methylpyridin-2-yl)benzoic acid. Used for the treatment of cystic fibrosis. lumacaftor: a corrector of CF transmembrane conductance regulator (CTFR); structure in first source | aromatic amide; benzodioxoles; benzoic acids; cyclopropanes; organofluorine compound; pyridines | CFTR potentiator; orphan drug |