8.A.160.1.5
Kelch-like protein 3, KLHL3, of 587 aas and 0-2 N-terminal TMSs. Substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin ligase
complex that acts as a regulator of ion transport in the distal nephron (Furukawa et al. 2003, Wu and Peng 2013). The
BCR(KLHL3) complex acts by mediating ubiquitination of WNK4, an
inhibitor of potassium channel KCNJ1, leading to WNK4 degradation (Ohta et al. 2013, Wu and Peng 2013). The
BCR(KLHL3) complex also mediates ubiquitination and degradation of
CLDN8, a tight-junction protein required for paracellular chloride
transport in the kidney. It is involved in chloride-sensitive signaling that turns the potassium switch on (Hoorn and de Baaij 2022).
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| Accession Number: | Q9UH77 |
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| Protein Name: | Kelch-like protein 3 |
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| Length: | 587 |
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| Molecular Weight: | 64970.00 |
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| Species: | Homo sapiens (Human) [9606] |
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| Location1 / Topology2 / Orientation3: |
Cytoplasm1 |
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| Substrate |
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1: MEGESVKLSS QTLIQAGDDE KNQRTITVNP AHMGKAFKVM NELRSKQLLC DVMIVAEDVE
61: IEAHRVVLAA CSPYFCAMFT GDMSESKAKK IEIKDVDGQT LSKLIDYIYT AEIEVTEENV
121: QVLLPAASLL QLMDVRQNCC DFLQSQLHPT NCLGIRAFAD VHTCTDLLQQ ANAYAEQHFP
181: EVMLGEEFLS LSLDQVCSLI SSDKLTVSSE EKVFEAVISW INYEKETRLE HMAKLMEHVR
241: LPLLPRDYLV QTVEEEALIK NNNTCKDFLI EAMKYHLLPL DQRLLIKNPR TKPRTPVSLP
301: KVMIVVGGQA PKAIRSVECY DFEEDRWDQI AELPSRRCRA GVVFMAGHVY AVGGFNGSLR
361: VRTVDVYDGV KDQWTSIASM QERRSTLGAA VLNDLLYAVG GFDGSTGLAS VEAYSYKTNE
421: WFFVAPMNTR RSSVGVGVVE GKLYAVGGYD GASRQCLSTV EQYNPATNEW IYVADMSTRR
481: SGAGVGVLSG QLYATGGHDG PLVRKSVEVY DPGTNTWKQV ADMNMCRRNA GVCAVNGLLY
541: VVGGDDGSCN LASVEYYNPV TDKWTLLPTN MSTGRSYAGV AVIHKSL