2.A.49.2.12 Chloride (bromide; iodide) channel-2, CLC-2, CLC2, CLCN2; 94% identical to the rat orthologue, 2.A.49.2.6. Hyperpolarization
activates CLC-2 mainly by driving intracellular anions into the channel pores. Protonation
by extracellular H+ plays a minor role in dislodging the glutamate gate (De Jesús-Pérez et al. 2016). The absence of ClC-2 results in less differentiated colonic crypts and
increased tumorigenicity associated with colitis via dysregulation (Jin et al. 2018). Biallelic CLCN2 mutations cause retinal
degeneration by impairing retinal pigment epithelium phagocytosis and
chloride channel function (Xu et al. 2023).
|
Accession Number: | P51788 |
Protein Name: | Chloride channel protein 2 |
Length: | 898 |
Molecular Weight: | 98535.00 |
Species: | Homo sapiens (Human) [9606] |
Number of TMSs: | 10 |
Location1 / Topology2 / Orientation3: |
Cell membrane1 / Multi-pass membrane protein2 |
Substrate |
chloride, bromide, iodide |
---|
1: MAAAAAEEGM EPRALQYEQT LMYGRYTQDL GAFAKEEAAR IRLGGPEPWK GPPSSRAAPE
61: LLEYGRSRCA RCRVCSVRCH KFLVSRVGED WIFLVLLGLL MALVSWVMDY AIAACLQAQQ
121: WMSRGLNTSI LLQYLAWVTY PVVLITFSAG FTQILAPQAV GSGIPEMKTI LRGVVLKEYL
181: TLKTFIAKVI GLTCALGSGM PLGKEGPFVH IASMCAALLS KFLSLFGGIY ENESRNTEML
241: AAACAVGVGC CFAAPIGGVL FSIEVTSTFF AVRNYWRGFF AATFSAFIFR VLAVWNRDEE
301: TITALFKTRF RLDFPFDLQE LPAFAVIGIA SGFGGALFVY LNRKIVQVMR KQKTINRFLM
361: RKRLLFPALV TLLISTLTFP PGFGQFMAGQ LSQKETLVTL FDNRTWVRQG LVEELEPPST
421: SQAWNPPRAN VFLTLVIFIL MKFWMSALAT TIPVPCGAFM PVFVIGAAFG RLVGESMAAW
481: FPDGIHTDSS TYRIVPGGYA VVGAAALAGA VTHTVSTAVI VFELTGQIAH ILPVMIAVIL
541: ANAVAQSLQP SLYDSIIRIK KLPYLPELGW GRHQQYRVRV EDIMVRDVPH VALSCTFRDL
601: RLALHRTKGR MLALVESPES MILLGSIERS QVVALLGAQL SPARRRQHMQ ERRATQTSPL
661: SDQEGPPTPE ASVCFQVNTE DSAFPAARGE THKPLKPALK RGPSVTRNLG ESPTGSAESA
721: GIALRSLFCG SPPPEAASEK LESCEKRKLK RVRISLASDA DLEGEMSPEE ILEWEEQQLD
781: EPVNFSDCKI DPAPFQLVER TSLHKTHTIF SLLGVDHAYV TSIGRLIGIV TLKELRKAIE
841: GSVTAQGVKV RPPLASFRDS ATSSSDTETT EVHALWGPHS RHGLPREGSP SDSDDKCQ