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2.A.49.3.4
CLC-6 or CLCN6, Cl-:H+ late endosomal/lysosomal antiporter (43% identical to CLC-7) and 99% identical to the human ortholog (P51797). Cl- > I-; mutating the gating glutamate yielded ohmic anion conductance (Neagoe et al., 2010). A recurrent gain-of-function mutation in CLCN6, encoding the ClC-6 Cl-/H+-exchanger, causes early-onset neurodegeneration (Polovitskaya et al. 2020). Loss of ClC-6 function reduces Golgi calcium stores, which may play a role in vascular contraction and relaxation signaling in vascular smooth muscle cells. Thus, ClC-6 may modulate blood pressure by regulating Golgi calcium reserves, which in turn contribute to vascular smooth muscle function (Klemens et al. 2021).

Accession Number:O35454
Protein Name:Chloride transport protein 6
Length:870
Molecular Weight:96980.00
Species:mus musculus (Mouse) [10090]
Number of TMSs:8
Location1 / Topology2 / Orientation3: Endosome membrane1 / Multi-pass membrane protein2
Substrate chloride, bromide, iodide, hydron, nitric acid

Cross database links:

RefSeq: NP_036059.1   
Entrez Gene ID: 26372   
Pfam: PF00571    PF00654   
KEGG: mmu:26372   

Gene Ontology

GO:0010008 C:endosome membrane
GO:0016021 C:integral to membrane
GO:0015297 F:antiporter activity
GO:0005524 F:ATP binding
GO:0005247 F:voltage-gated chloride channel activity
GO:0006821 P:chloride transport
GO:0055085 P:transmembrane transport

References (2)

[1] “Alternative splicing of ClC-6 (a member of the ClC chloride-channel family) transcripts generates three truncated isoforms one of which, ClC-6c, is kidney-specific.”  Eggermont J.et.al.   9224655
[2] “Lysosomal storage disease upon disruption of the neuronal chloride transport protein ClC-6.”  Poet M.et.al.   16950870

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Predict TMSs (Predict number of transmembrane segments)
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FASTA formatted sequence 
1:	MAGCRGSVCC CCRWCCCCGE RESRTPEELT ILGETQEEED EILPRKDYES LDYDRCINDP 
61:	YLEVLETMDN KKGRRYEAVK WMVVFAIGVC TGLVGLFVDF SVRLFTQLKF GVVQTSVEEC 
121:	SQKGCLALSL LELLGFNLTF VFLASLLVLI EPVAAGSGIP EIKCYLNGVK VPGIVRLRTL 
181:	LCKVFGVLFS VSGGLFVGKE GPMIHSGAVV GAGLPQFQSI SLRKIQFNFP YFRSDRDKRD 
241:	FVSAGAAAGV AAAFGAPIGG TLFSLEEGSS FWNQGLTWKV LFCSMSATFT LNFFRSGIQF 
301:	GSWGSFQLPG LLNFGEFKCS DSDKKCHLWT AMDLGFFVVM GVIGGLLGAT FNCLNKRLAK 
361:	YRMRNVHPKP KLVRVLESLL VSLVTTVVVF VASMVLGECR QMSSTSQTGN GSFQLQVTSE 
421:	DVNSTIKAFF CPNDTYNDMA TLFFNSQESA ILQLFHQDGT FSPVTLALFF ILYFLLACWT 
481:	FGTSVPSGLF VPSLLCGAAF GRLVANVLKS YIGLGHLYSG TFALIGAAAF LGGVVRMTIS 
541:	LTVILIESTN EITYGLPIMV TLMVAKWTGD LFNKGIYDVH IGLRGVPLLE WETDVEMDKL 
601:	RASDIMEPNL TYVYPHTRIQ SLVSILRTTV HHAFPVVTEN RGNEKEFMKG NQLISNNIKF 
661:	KKSSILTRAG EQRKRGQSMK SYPSSELRNV CDEHVASEEP AEKEDLLQQM LERRYTPYPN 
721:	LYPDQSPSED WTMEERFRPL TFHGLVLRSQ LVTLLVRGVC YSESQSSASQ PRLSYAEMAE 
781:	DYPRYPDIHD LDLTLLNPRM IVDVTPYMNP SPFTVSPNTH VSQVFNLFRT MGLRHLPVVN 
841:	AVGEIVGIIT RHNLTNEFLQ ARLRQHYQTL