ID FXL13_HUMAN Reviewed; 735 AA. AC Q8NEE6; C9J565; C9J566; Q6UVW7; Q6UVW8; Q75MN5; Q86UJ5; Q8N7Y4; Q8TCL2; AC Q8WUF9; Q8WUG0; DT 15-MAR-2005, integrated into UniProtKB/Swiss-Prot. DT 02-NOV-2010, sequence version 3. DT 10-JUN-2026, entry version 177. DE RecName: Full=F-box and leucine-rich repeat protein 13 {ECO:0000303|PubMed:29348145}; DE AltName: Full=Dynein regulatory complex subunit 6; DE AltName: Full=F-box/LRR-repeat protein 13; GN Name=FBXL13; Synonyms=DRC6 {ECO:0000250|UniProtKB:Q8CDU4}, FBL13; OS Homo sapiens (Human). OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; OC Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; OC Homo. OX NCBI_TaxID=9606; RN [1] RP NUCLEOTIDE SEQUENCE [MRNA] (ISOFORMS 3 AND 4). RX PubMed=15820312; DOI=10.1016/j.ygeno.2005.01.013; RA Curtiss N.P., Bonifas J.M., Lauchle J.O., Balkman J.D., Kratz C.P., RA Emerling B.M., Green E.D., Le Beau M.M., Shannon K.M.; RT "Isolation and analysis of candidate myeloid tumor suppressor genes from a RT commonly deleted segment of 7q22."; RL Genomics 85:600-607(2005). RN [2] RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1), AND VARIANT MET-74. RC TISSUE=Testis; RX PubMed=14702039; DOI=10.1038/ng1285; RA Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., RA Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., RA Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S., RA Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., RA Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., RA Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., RA Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., RA Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., RA Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., RA Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., RA Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., RA Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., RA Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., RA Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., RA Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., RA Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., RA Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., RA Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., RA Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., RA Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., RA Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., RA Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., RA Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., RA Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., RA Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., RA Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., RA Isogai T., Sugano S.; RT "Complete sequencing and characterization of 21,243 full-length human RT cDNAs."; RL Nat. Genet. 36:40-45(2004). RN [3] RP NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]. RX PubMed=12853948; DOI=10.1038/nature01782; RA Hillier L.W., Fulton R.S., Fulton L.A., Graves T.A., Pepin K.H., RA Wagner-McPherson C., Layman D., Maas J., Jaeger S., Walker R., Wylie K., RA Sekhon M., Becker M.C., O'Laughlin M.D., Schaller M.E., Fewell G.A., RA Delehaunty K.D., Miner T.L., Nash W.E., Cordes M., Du H., Sun H., RA Edwards J., Bradshaw-Cordum H., Ali J., Andrews S., Isak A., Vanbrunt A., RA Nguyen C., Du F., Lamar B., Courtney L., Kalicki J., Ozersky P., RA Bielicki L., Scott K., Holmes A., Harkins R., Harris A., Strong C.M., RA Hou S., Tomlinson C., Dauphin-Kohlberg S., Kozlowicz-Reilly A., Leonard S., RA Rohlfing T., Rock S.M., Tin-Wollam A.-M., Abbott A., Minx P., Maupin R., RA Strowmatt C., Latreille P., Miller N., Johnson D., Murray J., RA Woessner J.P., Wendl M.C., Yang S.-P., Schultz B.R., Wallis J.W., RA Spieth J., Bieri T.A., Nelson J.O., Berkowicz N., Wohldmann P.E., RA Cook L.L., Hickenbotham M.T., Eldred J., Williams D., Bedell J.A., RA Mardis E.R., Clifton S.W., Chissoe S.L., Marra M.A., Raymond C., Haugen E., RA Gillett W., Zhou Y., James R., Phelps K., Iadanoto S., Bubb K., Simms E., RA Levy R., Clendenning J., Kaul R., Kent W.J., Furey T.S., Baertsch R.A., RA Brent M.R., Keibler E., Flicek P., Bork P., Suyama M., Bailey J.A., RA Portnoy M.E., Torrents D., Chinwalla A.T., Gish W.R., Eddy S.R., RA McPherson J.D., Olson M.V., Eichler E.E., Green E.D., Waterston R.H., RA Wilson R.K.; RT "The DNA sequence of human chromosome 7."; RL Nature 424:157-164(2003). RN [4] RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2), AND VARIANTS MET-74 AND RP GLY-692. RC TISSUE=Kidney, Liver, and Testis; RX PubMed=15489334; DOI=10.1101/gr.2596504; RG The MGC Project Team; RT "The status, quality, and expansion of the NIH full-length cDNA project: RT the Mammalian Gene Collection (MGC)."; RL Genome Res. 14:2121-2127(2004). RN [5] RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 285-735. RC TISSUE=Testis; RX PubMed=17974005; DOI=10.1186/1471-2164-8-399; RA Bechtel S., Rosenfelder H., Duda A., Schmidt C.P., Ernst U., RA Wellenreuther R., Mehrle A., Schuster C., Bahr A., Bloecker H., Heubner D., RA Hoerlein A., Michel G., Wedler H., Koehrer K., Ottenwaelder B., Poustka A., RA Wiemann S., Schupp I.; RT "The full-ORF clone resource of the German cDNA consortium."; RL BMC Genomics 8:399-399(2007). RN [6] RP FUNCTION, AND SUBCELLULAR LOCATION. RX PubMed=29348145; DOI=10.15252/embr.201744799; RA Fung E., Richter C., Yang H.B., Schaeffer I., Fischer R., Kessler B.M., RA Bassermann F., D'Angiolella V.; RT "FBXL13 directs the proteolysis of CEP192 to regulate centrosome RT homeostasis and cell migration."; RL EMBO Rep. 19:0-0(2018). CC -!- FUNCTION: Substrate-recognition component of the SCF (SKP1-CUL1-F-box CC protein)-type E3 ubiquitin ligase complex. Component of the nexin- CC dynein regulatory complex (N-DRC), a key regulator of ciliary/flagellar CC motility which maintains the alignment and integrity of the distal CC axoneme and regulates microtubule sliding in motile axonemes. CC Specifically targets CEP192 isoform 3 for ubiquitin-mediated CC proteolysis and thereby acts as a regulator of microtubule nucleation CC activity (PubMed:29348145). {ECO:0000250|UniProtKB:A8JHD7, CC ECO:0000269|PubMed:29348145}. CC -!- SUBUNIT: Component of the nexin-dynein regulatory complex (N-DRC). CC Directly interacts with SKP1 and CUL1. Interacts with DRC5. CC {ECO:0000250, ECO:0000250|UniProtKB:A8JHD7, CC ECO:0000250|UniProtKB:Q8CDU4}. CC -!- SUBCELLULAR LOCATION: Cytoplasm, cytoskeleton, flagellum axoneme CC {ECO:0000250|UniProtKB:A8JHD7}. Cytoplasm, cytoskeleton, microtubule CC organizing center, centrosome {ECO:0000269|PubMed:29348145}. CC -!- ALTERNATIVE PRODUCTS: CC Event=Alternative splicing; Named isoforms=4; CC Name=1; CC IsoId=Q8NEE6-1; Sequence=Displayed; CC Name=2; CC IsoId=Q8NEE6-2; Sequence=VSP_013004; CC Name=3; CC IsoId=Q8NEE6-3; Sequence=VSP_013005; CC Name=4; CC IsoId=Q8NEE6-4; Sequence=VSP_013003; CC -!- SIMILARITY: Belongs to the DRC6 family. {ECO:0000305}. CC -!- SEQUENCE CAUTION: CC Sequence=CAD28506.1; Type=Erroneous initiation; Note=Extended N-terminus.; Evidence={ECO:0000305}; CC --------------------------------------------------------------------------- CC Copyrighted by the UniProt Consortium, see https://www.uniprot.org/terms CC Distributed under the Creative Commons Attribution (CC BY 4.0) License CC --------------------------------------------------------------------------- DR EMBL; AY359238; AAR13262.1; -; mRNA. DR EMBL; AY359239; AAR13263.1; -; mRNA. DR EMBL; AK097537; BAC05092.1; -; mRNA. DR EMBL; AC005250; AAP22333.1; -; Genomic_DNA. DR EMBL; AC006477; AAS00354.1; -; Genomic_DNA. DR EMBL; AC073127; -; NOT_ANNOTATED_CDS; Genomic_DNA. DR EMBL; BC020572; AAH20572.2; -; mRNA. DR EMBL; BC020575; AAH20575.2; -; mRNA. DR EMBL; BC031285; AAH31285.1; -; mRNA. DR EMBL; AL713709; CAD28506.1; ALT_INIT; mRNA. DR CCDS; CCDS5726.1; -. [Q8NEE6-1] DR RefSeq; NP_001104508.1; NM_001111038.2. [Q8NEE6-3] DR RefSeq; NP_001274079.1; NM_001287150.2. [Q8NEE6-2] DR RefSeq; NP_659469.3; NM_145032.3. [Q8NEE6-1] DR RefSeq; XP_011514232.1; XM_011515930.3. [Q8NEE6-1] DR RefSeq; XP_016867342.1; XM_017011853.2. [Q8NEE6-1] DR AlphaFoldDB; Q8NEE6; -. DR SMR; Q8NEE6; -. DR BioGRID; 128792; 42. DR ComplexPortal; CPX-2241; SCF E3 ubiquitin ligase complex, FBXL13 variant. DR ComplexPortal; CPX-8086; Nexin-dynein regulatory complex. DR FunCoup; Q8NEE6; 130. DR IntAct; Q8NEE6; 5. DR MINT; Q8NEE6; -. DR NDEx; IQUERY-CP-FBXL13; 1 NDEx IQuery Curated Pathway. DR STRING; 9606.ENSP00000321927; -. DR GlyGen; Q8NEE6; 1 site, 1 O-linked glycan (1 site). DR iPTMnet; Q8NEE6; -. DR PhosphoSitePlus; Q8NEE6; -. DR SwissPalm; Q8NEE6; -. DR BioMuta; FBXL13; -. DR DMDM; 311033450; -. DR jPOST; Q8NEE6; -. DR MassIVE; Q8NEE6; -. DR PaxDb; 9606-ENSP00000321927; -. DR PeptideAtlas; Q8NEE6; -. DR ProteomicsDB; 73152; -. [Q8NEE6-1] DR ProteomicsDB; 73153; -. [Q8NEE6-2] DR ProteomicsDB; 73154; -. [Q8NEE6-3] DR ProteomicsDB; 73155; -. [Q8NEE6-4] DR Antibodypedia; 31140; 105 antibodies from 21 providers. DR DNASU; 222235; -. DR Ensembl; ENST00000313221.8; ENSP00000321927.4; ENSG00000161040.20. [Q8NEE6-1] DR GeneID; 222235; -. DR KEGG; hsa:222235; -. DR UCSC; uc003vaq.3; human. [Q8NEE6-1] DR AGR; HGNC:21658; -. DR ClinPGx; PA134938720; -. DR CTD; 222235; -. DR DisGeNET; 222235; -. DR GeneCards; FBXL13; -. DR HGNC; HGNC:21658; FBXL13. DR HPA; ENSG00000161040; Tissue enhanced (stomach, testis). DR MIM; 609080; gene. DR OpenTargets; ENSG00000161040; -. DR VEuPathDB; HostDB:ENSG00000161040; -. DR eggNOG; KOG1947; Eukaryota. DR GeneTree; ENSGT00940000160224; -. DR HOGENOM; CLU_012282_0_0_1; -. DR InParanoid; Q8NEE6; -. DR OMA; ARCHYLH; -. DR OrthoDB; 61560at2759; -. DR PAN-GO; Q8NEE6; 2 GO annotations based on evolutionary models. DR PhylomeDB; Q8NEE6; -. DR PathwayCommons; Q8NEE6; -. DR Reactome; R-HSA-8951664; Neddylation. DR Reactome; R-HSA-983168; Antigen processing: Ubiquitination & Proteasome degradation. DR SignaLink; Q8NEE6; -. DR Agora; ENSG00000161040; -. DR BioGRID-ORCS; 222235; 18 hits in 1195 CRISPR screens. DR ChiTaRS; FBXL13; human. DR GenomeRNAi; 222235; -. DR Pharos; Q8NEE6; Tbio. DR PRO; PR:Q8NEE6; -. DR Proteomes; UP000005640; Chromosome 7. DR RNAct; Q8NEE6; protein. DR Bgee; ENSG00000161040; Expressed in sperm and 110 other cell types or tissues. DR ExpressionAtlas; Q8NEE6; baseline and differential. DR GO; GO:0005930; C:axoneme; NAS:ComplexPortal. DR GO; GO:0005813; C:centrosome; IEA:UniProtKB-SubCell. DR GO; GO:0005829; C:cytosol; TAS:Reactome. DR GO; GO:0019005; C:SCF ubiquitin ligase complex; IBA:GO_Central. DR GO; GO:0003341; P:cilium movement; NAS:ComplexPortal. DR GO; GO:0030317; P:flagellated sperm motility; NAS:ComplexPortal. DR GO; GO:0010824; P:regulation of centrosome duplication; NAS:ComplexPortal. DR GO; GO:0031146; P:SCF-dependent proteasomal ubiquitin-dependent protein catabolic process; IBA:GO_Central. DR CDD; cd22124; F-box_FBXL13; 1. DR FunFam; 3.80.10.10:FF:000134; F-box and leucine rich repeat protein 13; 1. DR FunFam; 3.80.10.10:FF:000266; F-box and leucine rich repeat protein 13; 1. DR FunFam; 3.80.10.10:FF:000291; F-box and leucine rich repeat protein 13; 1. DR Gene3D; 3.80.10.10; Ribonuclease Inhibitor; 3. DR InterPro; IPR036047; F-box-like_dom_sf. DR InterPro; IPR001810; F-box_dom. DR InterPro; IPR057207; FBXL15_LRR. DR InterPro; IPR006553; Leu-rich_rpt_Cys-con_subtyp. DR InterPro; IPR032675; LRR_dom_sf. DR PANTHER; PTHR13318:SF240; F-BOX AND LEUCINE-RICH REPEAT PROTEIN 13; 1. DR PANTHER; PTHR13318; PARTNER OF PAIRED, ISOFORM B-RELATED; 1. DR Pfam; PF12937; F-box-like; 1. DR Pfam; PF25372; LRR_FBXL15; 2. DR SMART; SM00367; LRR_CC; 14. DR SUPFAM; SSF81383; F-box domain; 1. DR SUPFAM; SSF52047; RNI-like; 2. DR PROSITE; PS50181; FBOX; 1. PE 1: Evidence at protein level; KW Alternative splicing; Cell projection; Cilium; Cytoplasm; Cytoskeleton; KW Flagellum; Leucine-rich repeat; Proteomics identification; KW Reference proteome; Repeat; Ubl conjugation pathway. FT CHAIN 1..735 FT /note="F-box and leucine-rich repeat protein 13" FT /id="PRO_0000119859" FT DOMAIN 152..198 FT /note="F-box" FT /evidence="ECO:0000255|PROSITE-ProRule:PRU00080" FT REPEAT 230..254 FT /note="LRR 1" FT REPEAT 255..280 FT /note="LRR 2" FT REPEAT 281..305 FT /note="LRR 3" FT REPEAT 306..333 FT /note="LRR 4" FT REPEAT 334..359 FT /note="LRR 5" FT REPEAT 360..385 FT /note="LRR 6" FT REPEAT 386..406 FT /note="LRR 7" FT REPEAT 410..435 FT /note="LRR 8" FT REPEAT 436..460 FT /note="LRR 9" FT REPEAT 461..488 FT /note="LRR 10" FT REPEAT 489..514 FT /note="LRR 11" FT REPEAT 515..538 FT /note="LRR 12" FT REPEAT 539..563 FT /note="LRR 13" FT REPEAT 564..589 FT /note="LRR 14" FT REPEAT 590..615 FT /note="LRR 15" FT REPEAT 616..641 FT /note="LRR 16" FT REPEAT 642..667 FT /note="LRR 17" FT REGION 682..703 FT /note="Disordered" FT /evidence="ECO:0000256|SAM:MobiDB-lite" FT COMPBIAS 682..692 FT /note="Polar residues" FT /evidence="ECO:0000256|SAM:MobiDB-lite" FT VAR_SEQ 337..618 FT /note="Missing (in isoform 4)" FT /evidence="ECO:0000303|PubMed:15820312" FT /id="VSP_013003" FT VAR_SEQ 546..573 FT /note="Missing (in isoform 2)" FT /evidence="ECO:0000303|PubMed:15489334" FT /id="VSP_013004" FT VAR_SEQ 574..618 FT /note="Missing (in isoform 3)" FT /evidence="ECO:0000303|PubMed:15820312" FT /id="VSP_013005" FT VARIANT 74 FT /note="I -> M (in dbSNP:rs7805950)" FT /evidence="ECO:0000269|PubMed:14702039, FT ECO:0000269|PubMed:15489334" FT /id="VAR_021480" FT VARIANT 313 FT /note="G -> A (in dbSNP:rs17135923)" FT /id="VAR_031426" FT VARIANT 535 FT /note="I -> V (in dbSNP:rs17135873)" FT /id="VAR_031427" FT VARIANT 565 FT /note="Y -> C (in dbSNP:rs17136118)" FT /id="VAR_031428" FT VARIANT 692 FT /note="D -> G (in dbSNP:rs17852944)" FT /evidence="ECO:0000269|PubMed:15489334" FT /id="VAR_031429" FT CONFLICT 110 FT /note="I -> V (in Ref. 1; AAR13262)" FT /evidence="ECO:0000305" FT CONFLICT 337 FT /note="I -> V (in Ref. 1; AAR13263)" FT /evidence="ECO:0000305" FT CONFLICT 496 FT /note="S -> F (in Ref. 5; CAD28506)" FT /evidence="ECO:0000305" FT CONFLICT 674 FT /note="K -> E (in Ref. 1; AAR13263)" FT /evidence="ECO:0000305" SQ SEQUENCE 735 AA; 83924 MW; 27F92DC4693C2581 CRC64; MTPELMIKAC SFYTGHLVKT HFCTWRDIAR TNENVVLAEK MNRAVTCYNF RLQKSVFHHW HSYMEDQKEK LKNILLRIQQ IIYCHKLTII LTKWRNTARH KSKKKEDELI LKHELQLKKW KNRLILKRAA AEESNFPERS SSEVFLVDET LKCDISLLPE RAILQIFFYL SLKDVIICGQ VNHAWMLMTQ LNSLWNAIDF SSVKNVIPDK YIVSTLQRWR LNVLRLNFRG CLLRPKTFRS VSHCRNLQEL NVSDCPTFTD ESMRHISEGC PGVLCLNLSN TTITNRTMRL LPRHFHNLQN LSLAYCRRFT DKGLQYLNLG NGCHKLIYLD LSGCTQISVQ GFRYIANSCT GIMHLTINDM PTLTDNCVKA LVEKCSRITS LVFTGAPHIS DCTFRALSAC KLRKIRFEGN KRVTDASFKF IDKNYPNLSH IYMADCKGIT DSSLRSLSPL KQLTVLNLAN CVRIGDMGLK QFLDGPASMR IRELNLSNCV RLSDASVMKL SERCPNLNYL SLRNCEHLTA QGIGYIVNIF SLVSIDLSGT DISNEGLNVL SRHKKLKELS VSECYRITDD GIQAFCKSSL ILEHLDVSYC SQLSDMIIKA LAIYCINLTS LSIAGCPKIT DSAMEMLSAK CHYLHILDIS GCVLLTDQIL EDLQIGCKQL RILKMQYCTN ISKKAAQRMS SKVQQQEYNT NDPPRWFGYD REGNPVTELD NITSSKGALE LTVKKSTYSS EDQAA //