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NPHP4

Gene
nphp4
Protein
Nephrocystin-4
Organism
Xenopus laevis
Length
1430 amino acids
Function
Involved in the organization of apical junctions (By similarity). Required for building functional cilia. Involved in the organization of the subapical actin network in multiciliated epithelial cells. Seems to recruit int to basal bodies of motile cilia which subsequently interacts with actin-modifying proteins such as daam1 (PubMed:26644512). May downregulate the canonical Wnt pathway and promote the Wnt-PCP pathway. Acts as negative regulator of the hippo pathway (By similarity).
Similarity
Belongs to the NPHP4 family.
Mass
161.486 kDa
Sequence
MKDWENIFSQSHIIPPHKQRCKHAPSEKAGYQICIETLEGTPFRQGVLERSVEVSCQLRMTLFDSTYHHFFGRTWKSSTKPIKAIPGKASKILFNEPIYFYTTLIDPSIMAVIEVVALSKKQGGSKQELSCGFGIMSLFSHQLEDDSSIQSGRLKLYYGTPRSLLHPTLKDPNELNQHMTLIENTCIQYSVRPHKVLPTVAHLFPENVLVSGSETIPGIMPSCDNSGDALKKPHVQKTVICYLDQICLFLYPTLEKFEEELTMLVNSDWLPKDDGNINGGSVSIQERRLHIGVHNGWVFVQKPQIVVVVPEAEMMRGHSTTSLKKKNIAQQKLSSTVQALVLRSRIRLPEMVNHHGFALVFLLEYVFSIPNGIHSKTPSAASSTSYMHMIRWASWTPSMEQGSADVALPLQGGPHHNPSNNLVYKMPPANMSSEEVQQVESGTVHFTFCAGSENQLEKTRDFAETTSKELIQSKKNVKMSNSKLVPETTFSPLESQVGPALSISQLTTSPRYPAISHSSKTAMQYIPSQLLPSPMAYQLSHAELPYASSITHLEADMSESHPNNSNGEHLQELLFSPVHAPIVAMGTQTGSTTSLLSRASLARLHSVEFPEILDCNNEVAEVVDPSNPVNFNLQREEADYLLCNEIVLQFLAFTRIHDGSTIWPESIFFTFQFYRFHHVTTPRLQLLQLESSDIATADPLTHVLVQINKDGSIKKGSPGFQIKYSVDPGFLKPGERKWFLRFLALQTLQIDIWDGESLLLIGSAAVEMKHLLRQGRTAVQVSHDLEVITTEYEQDLMVMSGDTAKQGTAKPIGVHTIIKGRLHMRMGNVGRPPEKKLKRRETLPPSNSRIITMHDGRTGFHGGSMLSNKSMNWKQSVCQAHKLADLDSELAAMLCSRMKEVSIAYQQTNSETDTTERRKKERMMAVRQQESQENANLRKSLIMAQHEERTQHTRDLQIIEAYRERTKPECISSMLNQAITSNYTVYATLGTAEFFEFELKNPYNIQYTVTIEIDSTDLRVITDTREWKHFKELTSTVTPIEENMFHVQPNTVIPQLYLRAKETVYIPFKYQTFCVDHTPMLQGPDAESLRKHSQLSQYKSNAMSSRRIKVSFKTSDGKLIAILQVNIEPQPHVVDQTFRFYHPELTFLKKSIRLPPWYTLPGAPVGTPGGEPDLQVRCSDINIICDSKKMGPGEPQDVFIKVAGGPSPQIKRFFIALYIDPWLAAPIQIWQIYVHSLKRVDVSCITGQLTQLSLVLRGTQVVRKVRAYSSHPEELKVDPDGVFVLPPNGIQDLHIGVRPQKAGSQFIYLNLVDVDQHQLVASWLVCVSCRKPVISKAFEIALPVGGEKGCNKRISYRNPYPTKKTYSLYTNRSDLLQFKENYFEVAAGETYSIGLRFAPSQTRGQEEILIFINDREDKNEETFCVKVRYE

Gene
NPHP4
Protein
Nephrocystin-4
Organism
Homo sapiens
Length
1426 amino acids
Function
Involved in the organization of apical junctions; the function is proposed to implicate a NPHP1-4-8 module (PubMed:19755384, PubMed:21565611). Does not seem to be strictly required for ciliogenesis (PubMed:21565611). Required for building functional cilia. Involved in the organization of the subapical actin network in multiciliated epithelial cells. Seems to recruit INT to basal bodies of motile cilia which subsequently interacts with actin-modifying proteins such as DAAM1 (By similarity). In cooperation with INVS may downregulate the canonical Wnt pathway and promote the Wnt-PCP pathway by regulating expression and subcellular location of disheveled proteins. Stabilizes protein levels of JADE1 and promotes its translocation to the nucleus leading to cooperative inhibition of canonical Wnt signaling (PubMed:21498478, PubMed:22654112). Acts as negative regulator of the hippo pathway by association with LATS1 and modifying LATS1-dependent phosphorylation and localization of WWTR1/TAZ (PubMed:21555462).
Similarity
Belongs to the NPHP4 family.
Mass
157.598 kDa
Sequence
MNDWHRIFTQNVLVPPHPQRARQPWKESTAFQCVLKWLDGPVIRQGVLEVLSEVECHLRVSFFDVTYRHFFGRTWKTTVKPTKRPPSRIVFNEPLYFHTSLNHPHIVAVVEVVAEGKKRDGSLQTLSCGFGILRIFSNQPDSPISASQDKRLRLYHGTPRALLHPLLQDPAEQNRHMTLIENCSLQYTLKPHPALEPAFHLLPENLLVSGLQQIPGLLPAHGESGDALRKPRLQKPITGHLDDLFFTLYPSLEKFEEELLELHVQDHFQEGCGPLDGGALEILERRLRVGVHNGLGFVQRPQVVVLVPEMDVALTRSASFSRKVVSSSKTSSGSQALVLRSRLRLPEMVGHPAFAVIFQLEYVFSSPAGVDGNAASVTSLSNLACMHMVRWAVWNPLLEADSGRVTLPLQGGIQPNPSHCLVYKVPSASMSSEEVKQVESGTLRFQFSLGSEEHLDAPTEPVSGPKVERRPSRKPPTSPSSPPAPVPRVLAAPQNSPVGPGLSISQLAASPRSPTQHCLARPTSQLPHGSQASPAQAQEFPLEAGISHLEADLSQTSLVLETSIAEQLQELPFTPLHAPIVVGTQTRSSAGQPSRASMVLLQSSGFPEILDANKQPAEAVSATEPVTFNPQKEESDCLQSNEMVLQFLAFSRVAQDCRGTSWPKTVYFTFQFYRFPPATTPRLQLVQLDEAGQPSSGALTHILVPVSRDGTFDAGSPGFQLRYMVGPGFLKPGERRCFARYLAVQTLQIDVWDGDSLLLIGSAAVQMKHLLRQGRPAVQASHELEVVATEYEQDNMVVSGDMLGFGRVKPIGVHSVVKGRLHLTLANVGHPCEQKVRGCSTLPPSRSRVISNDGASRFSGGSLLTTGSSRRKHVVQAQKLADVDSELAAMLLTHARQGKGPQDVSRESDATRRRKLERMRSVRLQEAGGDLGRRGTSVLAQQSVRTQHLRDLQVIAAYRERTKAESIASLLSLAITTEHTLHATLGVAEFFEFVLKNPHNTQHTVTVEIDNPELSVIVDSQEWRDFKGAAGLHTPVEEDMFHLRGSLAPQLYLRPHETAHVPFKFQSFSAGQLAMVQASPGLSNEKGMDAVSPWKSSAVPTKHAKVLFRASGGKPIAVLCLTVELQPHVVDQVFRFYHPELSFLKKAIRLPPWHTFPGAPVGMLGEDPPVHVRCSDPNVICETQNVGPGEPRDIFLKVASGPSPEIKDFFVIIYSDRWLATPTQTWQVYLHSLQRVDVSCVAGQLTRLSLVLRGTQTVRKVRAFTSHPQELKTDPKGVFVLPPRGVQDLHVGVRPLRAGSRFVHLNLVDVDCHQLVASWLVCLCCRQPLISKAFEIMLAAGEGKGVNKRITYTNPYPSRRTFHLHSDHPELLRFREDSFQVGGGETYTIGLQFAPSQRVGEEEILIYINDHEDKNEEAFCVKVIYQ

Gene
Nphp4
Protein
Nephrocystin-4
Organism
Mus musculus
Length
1425 amino acids
Function
Involved in the organization of apical junctions; the function is proposed to implicate a NPHP1-4-8 module. Does not seem to be strictly required for ciliogenesis (By similarity). Required for building functional cilia. Involved in the organization of the subapical actin network in multiciliated epithelial cells. Seems to recruit INT to basal bodies of motile cilia which subsequently interacts with actin-modifying proteins such as DAAM1 (By similarity). In cooperation with INVS may downregulate the canonical Wnt pathway and promote the Wnt-PCP pathway by regulating expression and subcellular location of disheveled proteins. Stabilizes protein levels of JADE1 and promotes its translocation to the nucleus leading to cooperative inhibition of canonical Wnt signaling (By similarity). Acts as negative regulator of the hippo pathway by association with LATS1 and modifying LATS1-dependent phosphorylation and localization of WWTR1/TAZ (By similarity).
Similarity
Belongs to the NPHP4 family.
Mass
157.269 kDa
Sequence
MGDWHRAFTQNTLVPPHPQRARQLGKESTAFQCILKWLDGPLIKQGILDMLSELECHLRVTLFDVTYKHFFGRTWKTTVKPTNQPSKQPPRITFNEPLYFHTTLSHPSIVAVVEVVTEGRKRDGTLQLLSCGFGILRIFGNKPESPTSAAQDKRLRLYHGTPRALLHPLLQDPIEQNKFMRLMENCSLQYTLKPHPPLEPAFHLLPENLLVSGFQQIPGLLPPHGDTGDALRKPRFQKPTTWHLDDLFFTLYPSLEKFEEELVQLLISDREGVGLLDSGTLEVLERRLHVCVHNGLGFVQRPQVVVLVPEMDVALTRSASFSRKISASSKNSSGNQALVLRSHLRLPEMVSHPAFAIVFQLEYVFNSPSGADGGASSPTSISSVACMHMVRWAVWNPDLEVGPGKVTLPLQGGVQQNPSRCLVYKVPSASMSSEEVKQVESGTIQFQFSLSSDGPTEHANGPRVGRRSSRKMPASPSGTPAPAARDLAATQDSPVGPGLSLSQLTASPLSPALQSSSKPPLQPPDSSQSPEGPQLQAESVLESRVSHLEADLSQPASLQGTPAVEHLQELPFTPLHAPIVVGAQTRSSRSQLSRAAMVLLQSSGFPEILDASQQPVEAVNPIDPVRFNPQKEESDCLRGNEIVLQFLAFSRAAQDCPGTPWPQTVYFTFQFYRFPPETTPRLQLVKLDGTGKSGSGSLSHILVPINKDGSFDAGSPGLQLRYMVDPGFLKPGEQRWFAHYLAAQTLQVDVWDGDSLLLIGSAGVQMKHLLRQGRPAVQVSHELEVVATEYEQEMMAVSGDVAGFGSVKPIGVHTVVKGRLHLTLANVGHACEPRARGSNLLPPSRSRVISNDGASFFSGGSLLIPGGPKRKRVVQAQRLADVDSELAAMLLTHTRAGQGPQAAGQEADAVHKRKLERMRLVRLQEAGGDSDSRRISLLAQHSVRAQHSRDLQVIDAYRERTKAESIAGVLSQAITTHHTLYATLGTAEFFEFALKNPHNTQHTVAIEIDSPELSIILDSQEWRYFKEATGLHTPLEEDMFHLRGSLAPQLYLRPRETAHIPLKFQSFSVGPLAPTQAPAEVITEKDAESGPLWKCSAMPTKHAKVLFRVETGQLIAVLCLTVEPQPHVVDQVFRFYHPELTFLKKAIRLPPWHTLPGAPVGMPGEDPPVHVRCSDPNVICEAQNVGPGEPRDVFLKVASGPSPEIKDFFVVIYADRWLAVPVQTWQVCLHSLQRVDVSCVAGQLTRLSLVLRGTQTVRKVRAFTSHPQELKTDPAGVFVLPPHGVQDLHVGVRPRRAGSRFVHLNLVDIDYHQLVASWLVCLSCRQPLISKAFEITMAAGDEKGTNKRITYTNPYPSRRTYRLHSDRPELLRFKEDSFQVAGGETYTIGLRFLPSGSAGQEEILIYINDHEDKNEETFCVKVLYQ