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1.A.1.2.17
Potassium voltage-gated channel (KCNH) subfamily G member 3 (Voltage-gated potassium channel subunit Kv10.1) (Voltage-gated potassium channel subunit Kv6.3).  Splice variants have different properties and can activate cyclin-dependent protein kinases (Ramos Gomes et al. 2015). Control of transport (pore) function  by the voltage sensor may involve more than one mechanism (Tomczak et al. 2017).  The silent (non transporting) behaviour of Kv6.3 in the ER is caused by the C-terminal part of its sixth transmembrane domain that causes ER retention (Ottschytsch et al. 2005).  De novo missense variants in KCNH1 encoding Kv10.1 are responsible for two clinically recognisable phenotypes: Temple-Baraitser syndrome (TBS) and Zimmermann-Laband syndrome (ZLS) (Aubert Mucca et al. 2022). The clinical overlap between these two syndromes suggests that they belong to a spectrum of KCNH1-related encephalopathies. Affected patients have severe intellectual disability (ID) with or without epilepsy, hypertrichosis and distinctive features such as gingival hyperplasia and nail hypoplasia/aplasia (Aubert Mucca et al. 2022).

Accession Number:Q8R523
Protein Name:Potassium voltage-gated channel subfamily G member 3
Length:433
Molecular Weight:49291.00
Species:Rattus norvegicus (Rat) [10116]
Number of TMSs:7
Location1 / Topology2 / Orientation3: Cell membrane1 / Multi-pass membrane protein2
Substrate potassium(1+)

Cross database links:

Entrez Gene ID: 171011   
Pfam: PF00520    PF02214   
KEGG: rno:171011   

Gene Ontology

GO:0005783 C:endoplasmic reticulum
GO:0008076 C:voltage-gated potassium channel complex
GO:0005249 F:voltage-gated potassium channel activity
GO:0051260 P:protein homooligomerization

References (1)

[1] “Molecular cloning and characterization of Kv6.3, a novel modulatory subunit for voltage-gated K(+) channel Kv2.1.”  Sano Y.et.al.   11852086

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Predict TMSs (Predict number of transmembrane segments)
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FASTA formatted sequence
1:	MTFGRGGAAS VVLNVGGARY SLSRELLKDF PLRRVSRLHG CRSERDVLEV CDDYDRERNE 
61:	YFFDRHSEAF GFILLYVRGH GKLRFAPRMC ELSFYNEMIY WGLEGAHLEY CCQRRLDDRM 
121:	SDTHTFHAAE ELGREQPRPT GPEAAPSRRW LERMRRTFEE PTSSLAAQIL ASVSVVFVIV 
181:	SMVVLCASTL PDWRAAAADN RSLDDRSRYS ASPGREPSGI IEAICIGWFT AECIVRFIVS 
241:	KNKCEFVKRP LNIIDLLAIT PYYISVLMTV FTGENSQLQR AGVTLRVLRM MRIFWVIKLA 
301:	RHFIGLQTLG LTLKRCYREM VMLLVFICVA MAIFSALSQL LEHGLDLETS NKDFASIPAA 
361:	CWWVIISMTT VGYGDMYPIT VPGRILGGVC VVSGIVLLAL PITFIYHSFV QCYHELKFRS 
421:	ARYSRSLSAE FLN