8.A.160.1.6
Plankophilin-2, PKP2, of 881 aas and 1 central TMS. It is a component of desmosome cell-cell junctions (Todorovic et al. 2014). It is required for cardiac sodium current propagation and electrical synchrony
in cardiac myocytes, via ANK3 stabilization and modulation of
SCN5A/Nav1.5 localization to cell-cell junctions. It may regulate the localization of GJA1 to gap junctions in intercalated disks of the heart (Fidler et al. 2009). Exercise-induced dysregulation of the adrenergic response in a mouse model of PKP2-arrhythmogenic cardiomyopathy has been documented (van Opbergen et al. 2025).
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| Accession Number: | Q99959 |
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| Protein Name: | Plakophilin-2 |
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| Length: | 881 |
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| Molecular Weight: | 97415.00 |
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| Species: | Homo sapiens (Human) [9606] |
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| Number of TMSs: | 1 |
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| Location1 / Topology2 / Orientation3: |
Nucleus1 |
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| Substrate |
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1: MAAPGAPAEY GYIRTVLGQQ ILGQLDSSSL ALPSEAKLKL AGSSGRGGQT VKSLRIQEQV
61: QQTLARKGRS SVGNGNLHRT SSVPEYVYNL HLVENDFVGG RSPVPKTYDM LKAGTTATYE
121: GRWGRGTAQY SSQKSVEERS LRHPLRRLEI SPDSSPERAH YTHSDYQYSQ RSQAGHTLHH
181: QESRRAALLV PPRYARSEIV GVSRAGTTSR QRHFDTYHRQ YQHGSVSDTV FDSIPANPAL
241: LTYPRPGTSR SMGNLLEKEN YLTAGLTVGQ VRPLVPLQPV TQNRASRSSW HQSSFHSTRT
301: LREAGPSVAV DSSGRRAHLT VGQAAAGGSG NLLTERSTFT DSQLGNADME MTLERAVSML
361: EADHMLPSRI SAAATFIQHE CFQKSEARKR VNQLRGILKL LQLLKVQNED VQRAVCGALR
421: NLVFEDNDNK LEVAELNGVP RLLQVLKQTR DLETKKQITD HTVNLRSRNG WPGAVAHACN
481: PSTLGGQGGR ITRSGVRDQP DQHGLLWNLS SNDKLKNLMI TEALLTLTEN IIIPFSGWPE
541: GDYPKANGLL DFDIFYNVTG CLRNMSSAGA DGRKAMRRCD GLIDSLVHYV RGTIADYQPD
601: DKATENCVCI LHNLSYQLEA ELPEKYSQNI YIQNRNIQTD NNKSIGCFGS RSRKVKEQYQ
661: DVPMPEEKSN PKGVEWLWHS IVIRMYLSLI AKSVRNYTQE ASLGALQNLT AGSGPMPTSV
721: AQTVVQKESG LQHTRKMLHV GDPSVKKTAI SLLRNLSRNL SLQNEIAKET LPDLVSIIPD
781: TVPSTDLLIE TTASACYTLN NIIQNSYQNA RDLLNTGGIQ KIMAISAGDA YASNKASKAA
841: SVLLYSLWAH TELHHAYKKA QFKKTDFVNS RTAKAYHSLK D