5J1T,4TVS


Conserved Protein Domain Family
LAP1_C
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pfam05609: LAP1_C 
Lamina-associated polypeptide 1, AAA+ activator domain
Torsin-1A-interacting proteins 1 and 2 (TOIP 1/2), also known as LAP1 proteins (Lamina-associated polypeptide 1) are type 2 integral membrane proteins with a single membrane-spanning region of the inner nuclear membrane. These proteins interact with and activate Torsin A, an AAA+ ATPase localized to the endoplasmic reticulum (ER) through a perinuclear domain and forms a heterohexameric (LAP1-Torsin)3 ring that targets Torsin to the nuclear envelope. LAP1 has an atypical AAA+ fold and provides an arginine finger to the Torsin A active site to promote its ATPase activity. A single mutation in Torsin A causes early onset primary dystonia, a painful and severely disabling neuromuscular disease. This entry represents the C-terminal AAA+ activator domain of LAP1.
Links
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Statistics
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PSSM-Id: 461691
Aligned: 22 rows
Threshold Bit Score: 438.068
Created: 22-Mar-2022
Updated: 17-Oct-2022
Structure
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Program:
Drawing:
Aligned Rows:
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PubMed ReferencesClick to see Conserved Features Help
Sequence Alignment
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Format: Row Display: Color Bits: Type Selection: 
5J1T_B         6 SYYSSP-AQQVPKNpALEAFLAQFSQLEDKFPGQSSFLWQRGRKFLQKHLNASNP-TEPATIIFTAAREGRETLKCLSHH 83  human
XP_007480952 363 LYMNWPfATNVKTT-AVKEFQNQMKKLMKKYQSQDEKLWKRSQTFLEKHLNSSHPrPQPAILLLTAAQDAEETLRCLSEQ 441
ELK37141     248 SYYSSP-AQQVPPNpALEAFLAQFSQLQDTFPGQSAFLWQRGRKFLQKHLNASHP-TEPATIIFTAAREGRETLRCLSHR 325
ELK16221     235 SYYSSP-TQQAANNpALEAFLAHFSQLKDKFPGQSSFLWQRGRKFLQKHLNASNP-TEPATIIFTAAREGRETLKCLSHH 312
XP_010589283 370 WSSRNP---EVETT-AVQEFQNQMKQLMNKYRGQDEKLWKRSLTFLEKHLNSSIPrPQPAILLLTAARDAEEVLKCLSEQ 445
EHB02130     354 SFIHMP---GVETT-AVQMFQNQMKQLRNKYQGQDEKLWERSLMFLQKHLNSSHPrPQPAILLLTAAQDAEEALKCLSEQ 429
4TVS_A         1 gpgSTP---EVETT-AVQEFQNQMNQLKNKYQGQDEKLWKRSQTFLEKHLNSSHPrSQPAILLLTAARDAEEALRCLSEQ 76  human
XP_023366578 371 WFFYTP---EVETTaAVQEFQNQMNQLRSKYQGQDEKLWKRSLTLLEKHLNSSHPrPQPAVLLLTAARDAEEALKCLSEQ 447
ELK16218     259 WYPLTP---EAETT-AVKEFQNQMKQLMNKYQGQDEKLWKRSQTFLEKHLNSSQPrPQPAILLLTAARDAEEVLKCLSEQ 334
XP_008541823 366 WLSPTP---EVETT-AVQEFQNRMKQLMKKYQGQDEKLWKRSLTFLEKHLNSSQPrPQPAILLLTAAQDAEQALKCLSEQ 441

5J1T_B        84 VADAYTSSQKVSPIQIDGAGRTWQDSDTVKLLVDLELSYGFENGQKAAVVHHFESFPAGSTLIFYKYCDHENAAFKDVAL 163 human
XP_007480952 442 IADAYSSFYSVRAIRINGTGTSTQDSDYVKLEVDQKLSSGFKNGQNAAVVHRFESLPAGSTLIFYKYCDHENAAFKDVAL 521
ELK37141     326 VADAYTSSQKVSAIQIDGAGRALQDSDTVKLLVDMELSSGFEDGRKAAVVHRFESLPAGSTLIFYKYCDHENAAFKDVAL 405
ELK16221     313 VADAYTSSQKVSAIQIDGTGRTLQDSDVVKLSVDKLLSSGFEKGRKAAVVHRFESLPAGSTLIFYKYCDHENAAFKDVAL 392
XP_010589283 446 IADAYSSFRSVRAIRIDGTGKAAQDSDTVKLEVDQELSNGFRNGQNAAVVHRFESLPAGSTLIFYKYCDHENAAFKDVAL 525
EHB02130     430 IADAYSSFRSVRAIRIDGTGKAAQDSDVVKLEVDQELSNGFTNGQNAAVVHRLESLPAGSTLIFYKYCDHENAAFKDVAL 509
4TVS_A        77 IADAYSSFRSVRAIRIDGTDKATQDSDTVKLEVDQELSNGFKNGQNAAVVHRFESFPAGSTLIFYKYCDHENAAFKDVAL 156 human
XP_023366578 448 IADAYSSFWSVRAIRIDGAGKAAQDSDTVKLEVDQELSNGFKNGQNAAVVHRFESLPAGSTLIFYKYCDHENAAFKDVAL 527
ELK16218     335 IADAYSSFRSVRAIRIDGTGKATQDSDAVKLEVDQELTNGFRNGQNAAVVHRFESLPARSTLIFYKYCDHENAAFKDVAL 414
XP_008541823 442 IADAYSSVHSVRAIRIDGAGKATQDSDAVKLEVDQELSNGFRNGQNAAVVHRFESLPAGSTLIFYKYCDHENAAFKDVAL 521

5J1T_B       164 VLTVLLEEETLEASVGPRETEEKVRDLLWAKFTNSDTPTSFNHMDSDKLSGLWSRISHLVLPVQPVSSIEEQGCL 238 human
XP_007480952 522 ILTVLLEEKTLGTDLGLKEVEEKVRDFLKAKFTSSDTPNSYNHMDSDKLSGLWSRISHLVLPVQPENSLKSGVCL 596
ELK37141     406 VLTVLLEEDTLAASVGPRETEEKVRDLLWARFTNANTPSSFNRMDSDKLSGLWSRISHLVLPVQPVRAIEEQGCR 480
ELK16221     393 VLTVLLEEDTLEASVGPRETEEKVRDLLWARFTNSNTPSSFNHMDSDKLSGLWSRISHLVLPVQPISDPSITSQK 467
XP_010589283 526 VLTVLLEDETLETTLGLKEIEEKVRDFLRVKFTSSDTPDSYNHMDSDKLSGLWSRISHLVLPVQPENALKGGFCL 600
EHB02130     510 VLTVLLEEKTLGTSLGLKEIEEKVRDFLKVKFTSSSTPSSYNHMDPDKLNGLWSRISHLVLPVQPENALKRGICL 584
4TVS_A       157 VLTVLLEEETLGTSLGLKEVEEKVRDFLKVKFTNSNTPNSYNHMDPDKLNGLWSRISHLVLPVQPENALKRGICL 231 human
XP_023366578 528 VLTVLLEEETLGTSLGLKEIEEKVRDFLKVKFTNSDTPNSYNHMDPDKLNGLWSRISHLVLPVQPENTLKRGICL 602
ELK16218     415 VLTVLLEEETLGTSLGLKEIEEKVRDFLRVKFTNSDAPNSYKHMDPDKLSGLWSRISHLVLPVQPEDALKRGICL 489
XP_008541823 522 VLTVLLEEETLETSLGLKEIEEKVRDFLKVKFTNSDTPNSYKHMDPDKLSGLWSRISHLVLPIQPENALKGGICL 596
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