Literature summary for 5.6.1.6 extracted from

Jordan, I.K.; Kota, K.C.; Cui, G.; Thompson, C.H.; McCarty, N.A.
Evolutionary and functional divergence between the cystic fibrosis transmembrane conductance regulator and related ATP-binding cassette transporters (2008), Proc. Natl. Acad. Sci. USA, 105, 18865-18870.

Search for more literature for 5.6.1.6

Cloned(Commentary)

Cloned (Comment)	Organism
phylogenetic analysis and genotyping, expression of wild-type and mutant enzymes in Xenopus laevis oocytes	Homo sapiens

Protein Variants

Protein Variants	Comment	Organism
R352E	channels bearing the R352E mutation exhibit frequent transitions to subconductance levels	Homo sapiens
R352E/D993R	channels bearing the revertant mutation R352E/D993R, primarily exhibit transitions to the full conductance level	Homo sapiens
R352E/E1104R	channels bearing the R352E mutation, or the double mutant R352E/E1104R, exhibit frequent transitions to subconductance levels	Homo sapiens
R352X	charge-destroying mutations at R352 alter CFTR single channel behavior	Homo sapiens

Localization

Localization	Comment	Organism	GeneOntology No.	Textmining
membrane	-	Homo sapiens	16020	-

Natural Substrates/ Products (Substrates)

Natural Substrates	Organism	Comment (Nat. Sub.)	Natural Products	Comment (Nat. Pro.)	Rev.	Reac.
ATP + H2O + closed Cl- channel	Homo sapiens	CFTR acts as an ATP-dependent chloride channel. CFTR channel activity evolved, at least in part, by converting the conformational changes associated with binding and hydrolysis of ATP	ADP + phosphate + open Cl- channel	-	?

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	-	-	-

Substrates and Products (Substrate)

Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
ATP + H2O + closed Cl- channel	-	Homo sapiens	ADP + phosphate + open Cl- channel	-	?
ATP + H2O + closed Cl- channel	CFTR acts as an ATP-dependent chloride channel. CFTR channel activity evolved, at least in part, by converting the conformational changes associated with binding and hydrolysis of ATP	Homo sapiens	ADP + phosphate + open Cl- channel	-	?

Subunits

Subunits	Comment	Organism
More	comparison of CFTR protein sequences to those of other ABC transporters, structure analysis, overview. A residue, identified as being involved in CFTR functional divergence, by virtue of being both CFTR-specific and conserved among all CFTR orthologs, is R352 in the sixth transmembrane helix, TM6. R352 interacts with absolutely conserved D993 in TM9 to stabilize the open-channel state	Homo sapiens

Synonyms

Synonyms	Comment	Organism
CFTR	-	Homo sapiens
cystic fibrosis transmembrane conductance regulator	-	Homo sapiens
More	CFTR is a member of the ATP-binding cassette, ABC, transporter superfamily	Homo sapiens

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Release 2023.1 (January 2023)