Descriptions

The autoinhibited protein was predicted that may have potential autoinhibitory elements via cis-regPred.

Autoinhibitory domains (AIDs)

Target domain

Relief mechanism

Assay

cis-regPred

Accessory elements

No accessory elements

Autoinhibited structure

Activated structure

1 structures for Q2KJI7

Entry ID Method Resolution Chain Position Source
AF-Q2KJI7-F1 Predicted AlphaFoldDB

195 variants for Q2KJI7

Variant ID(s) Position Change Description Diseaes Association Provenance
rs133049052 11 R>G No EVA
rs516622484 30 G>D No EVA
rs800061505 58 D>N No EVA
rs444508421 76 K>E No EVA
rs462100796 82 K>N No EVA
rs441931299 83 N>D No EVA
rs473212427 96 E>A No EVA
rs443364118 183 K>R No EVA
rs455483634 253 S>P No EVA
rs466652118 258 M>I No EVA
rs435307962 258 M>L No EVA
rs452967438 261 T>P No EVA
rs432908912 262 V>E No EVA
rs464138942 267 F>V No EVA
rs450370560 269 L>V No EVA
rs481832679 271 T>P No EVA
rs469638959 280 G>D No EVA
rs480815004 282 T>K No EVA
rs460739661 294 E>D No EVA
rs440473962 299 V>G No EVA
rs720755595 311 V>M No EVA
rs450049589 391 R>C No EVA
rs481202282 396 L>I No EVA
rs461177181 399 K>* No EVA
rs440869155 400 N>H No EVA
rs478726052 410 I>L No EVA
rs470025061 424 G>V No EVA
rs458593393 426 S>R No EVA
rs438342718 428 Q>R No EVA
rs476016210 429 E>G No EVA
rs457474344 433 N>H No EVA
rs443939640 433 N>K No EVA
rs474993727 437 V>L No EVA
rs476578083 449 V>A No EVA
rs436393672 475 F>V No EVA
rs467650664 475 F>Y No EVA
rs447493202 476 I>V No EVA
rs433763890 477 G>* No EVA
rs461612540 477 G>A No EVA
rs472821046 478 P>Q No EVA
rs439042945 482 K>N No EVA
rs476668257 484 R>* No EVA
rs456636670 484 R>S No EVA
rs436328617 487 I>L No EVA
rs474254420 488 F>L No EVA
rs433907818 492 L>P No EVA
rs437775052 496 K>N No EVA
rs448903084 497 L>P No EVA
rs448903084 497 L>R No EVA
rs466558828 498 D>E No EVA
rs480247239 498 D>H No EVA
rs447936757 500 T>P No EVA
rs438983529 509 K>* No EVA
rs483211991 510 L>V No EVA
rs463048750 513 L>F No EVA
rs442985125 514 T>P No EVA
rs454072071 517 F>L No EVA
rs454072071 517 F>V No EVA
rs450756476 525 V>L No EVA
rs482024691 526 C>G No EVA
rs473167813 544 K>N No EVA
rs459575792 546 F>I No EVA
rs439450274 546 F>S No EVA
rs470650569 553 V>G No EVA
rs457037498 554 I>V No EVA
rs434776902 563 V>A No EVA
rs445948079 565 Q>P No EVA
rs432278207 566 P>T No EVA
rs449861227 567 E>A No EVA
rs470102828 567 E>Q No EVA
rs461045625 568 E>A No EVA
rs447332767 569 K>E No EVA
rs478638625 570 K>* No EVA
rs458402672 570 K>N No EVA
rs438382711 571 T>P No EVA
rs443771212 572 V>A No EVA
rs463900601 572 V>M No EVA
rs455009250 573 A>E No EVA
rs472565657 574 Y>D No EVA
rs452502932 577 A>E No EVA
rs432364284 579 H>P No EVA
rs470027009 579 H>Q No EVA
rs449948429 580 A>P No EVA
rs449948429 580 A>T No EVA
rs467575596 581 V>G No EVA
rs447312536 584 W>L No EVA
rs478482405 585 Y>D No EVA
rs458491867 585 Y>F No EVA
rs478482405 585 Y>H No EVA
rs444759545 586 L>M No EVA
rs444759545 586 L>V No EVA
rs482428782 587 E>G No EVA
rs443935243 588 H>P No EVA
rs475223216 589 A>P No EVA
rs461538306 590 D>A No EVA
rs461538306 590 D>V No EVA
rs441426924 592 L>I No EVA
rs472655430 592 L>P No EVA
rs438817866 594 K>R No EVA
rs457879066 595 V>G No EVA
rs444353216 596 S>C No EVA
rs482050907 598 I>T No EVA
rs441807167 603 G>E No EVA
rs473050330 606 Y>C No EVA
rs453026115 609 Y>C No EVA
rs453026115 609 Y>F No EVA
rs439222653 611 P>S No EVA
rs451894970 612 R>W No EVA
rs438312330 613 E>D No EVA
rs469688090 615 Y>S No EVA
rs455883909 618 T>P No EVA
rs435795272 621 Q>K No EVA
rs466858800 621 Q>R No EVA
rs446951498 624 D>H No EVA
rs477989689 628 M>R No EVA
rs464418251 629 T>A No EVA
rs450816406 630 L>M No EVA
rs481004624 633 R>H No EVA
rs482132207 637 E>* No EVA
rs462028081 639 F>I No EVA
rs441758696 646 G>R No EVA
rs479654935 647 A>P No EVA
rs459431367 648 Q>K No EVA
rs439362083 650 D>Y No EVA
rs456893130 651 L>F No EVA
rs455971036 654 V>G No EVA
rs435751063 655 T>S No EVA
rs453259661 656 Q>H No EVA
rs464507845 661 Q>E No EVA
rs472631057 662 I>T No EVA
rs452400840 666 G>C No EVA
rs432305157 666 G>V No EVA
rs467294538 667 M>I No EVA
rs456224094 667 M>L No EVA
rs436203168 667 M>T No EVA
rs447348660 668 N>H No EVA
rs478390580 668 N>T No EVA
rs447348660 668 N>Y No EVA
rs444845178 669 E>D No EVA
rs464831141 669 E>G No EVA
rs464831141 669 E>V No EVA
rs477727363 670 K>* No EVA
rs464065578 670 K>M No EVA
rs443752545 671 V>G No EVA
rs481706546 672 G>R No EVA
rs461439454 672 G>V No EVA
rs472567600 673 Q>E No EVA
rs472567600 673 Q>K No EVA
rs452502957 673 Q>L No EVA
rs438793838 674 I>M No EVA
rs456459579 676 F>V No EVA
rs436183860 677 D>Y No EVA
rs473832045 678 L>I No EVA
rs453664848 679 P>T No EVA
rs464918822 680 R>P No EVA
rs437601939 682 G>R No EVA
rs450408440 683 D>G No EVA
rs461574405 685 V>E No EVA
rs461574405 685 V>G No EVA
rs481590534 685 V>L No EVA
rs447899709 686 L>V No EVA
rs459059600 690 Y>* No EVA
rs479062462 690 Y>C No EVA
rs438778161 691 S>R No EVA
rs476690390 692 E>G No EVA
rs462804024 693 A>D No EVA
rs442747141 696 R>S No EVA
rs453752818 701 E>D No EVA
rs473921507 701 E>G No EVA
rs433755253 702 V>I No EVA
rs471268680 711 K>N No EVA
rs451423328 713 T>K No EVA
rs437582041 717 L>V No EVA
rs469029077 722 A>P No EVA
rs450282694 724 V>G No EVA
rs465958743 727 V>F No EVA
rs526299376 729 L>M No EVA
rs477113775 738 D>G No EVA
rs469899595 739 K>R No EVA
rs136070598 741 D>H No EVA
rs449560119 746 L>P No EVA
rs458560638 758 E>K No EVA
rs462262604 762 E>K No EVA
rs442313718 764 T>P No EVA
rs439785598 783 E>D No EVA
rs457479643 787 S>P No EVA
rs475027989 790 P>T No EVA
rs434632336 791 S>* No EVA
rs454805231 791 S>P No EVA
rs432160998 797 S>R No EVA
rs449737075 797 S>R No EVA
rs469855545 797 S>T No EVA
rs467168095 798 P>Q No EVA
rs480808403 798 P>S No EVA
rs447227729 799 V>G No EVA

No associated diseases with Q2KJI7

6 regional properties for Q2KJI7

Type Name Position InterPro Accession
domain Peptidase M41 563 - 744 IPR000642
domain AAA+ ATPase domain 341 - 480 IPR003593
domain ATPase, AAA-type, core 346 - 477 IPR003959
conserved_site ATPase, AAA-type, conserved site 448 - 466 IPR003960
domain Peptidase M41, FtsH extracellular 157 - 241 IPR011546
domain AAA ATPase, AAA+ lid domain 509 - 547 IPR041569

Functions

Description
EC Number
Subcellular Localization
  • Mitochondrion inner membrane ; Multi-pass membrane protein
PANTHER Family
PANTHER Subfamily
PANTHER Protein Class
PANTHER Pathway Category No pathway information available

2 GO annotations of cellular component

Name Definition
m-AAA complex Protease complex of the mitochondrial inner membrane that is involved in mitochondrial protein turnover and in processing of proteins imported into mitochondria.
mitochondrial inner membrane The inner, i.e. lumen-facing, lipid bilayer of the mitochondrial envelope. It is highly folded to form cristae.

6 GO annotations of molecular function

Name Definition
ATP binding Binding to ATP, adenosine 5'-triphosphate, a universally important coenzyme and enzyme regulator.
ATP hydrolysis activity Catalysis of the reaction: ATP + H2O = ADP + H+ phosphate. ATP hydrolysis is used in some reactions as an energy source, for example to catalyze a reaction or drive transport against a concentration gradient.
ATP-dependent peptidase activity Catalysis of the hydrolysis of peptide bonds, driven by ATP hydrolysis.
metalloendopeptidase activity Catalysis of the hydrolysis of internal, alpha-peptide bonds in a polypeptide chain by a mechanism in which water acts as a nucleophile, one or two metal ions hold the water molecule in place, and charged amino acid side chains are ligands for the metal ions.
metallopeptidase activity Catalysis of the hydrolysis of peptide bonds by a mechanism in which water acts as a nucleophile, one or two metal ions hold the water molecule in place, and charged amino acid side chains are ligands for the metal ions.
zinc ion binding Binding to a zinc ion (Zn).

17 GO annotations of biological process

Name Definition
axonogenesis De novo generation of a long process of a neuron, including the terminal branched region. Refers to the morphogenesis or creation of shape or form of the developing axon, which carries efferent (outgoing) action potentials from the cell body towards target cells.
calcium import into the mitochondrion A process in which a calcium ion (Ca2+) is transported from the cytosol into the mitochondrial matrix.
cristae formation The assembly of cristae, the inwards folds of the inner mitochondrial membrane.
membrane protein proteolysis The proteolytic cleavage of a transmembrane protein leading to the release of its intracellular or ecto-domains.
mitochondrial calcium ion homeostasis Any process involved in the maintenance of an internal steady state of calcium ions within the cytoplasm of a cell or between mitochondria and their surroundings.
mitochondrial fusion Merging of two or more mitochondria within a cell to form a single compartment.
mitochondrial protein processing The peptide cleavage of mitochondrial proteins, including cleavage contributing to their import.
muscle cell development The process whose specific outcome is the progression of a muscle cell over time, from its formation to the mature structure. Muscle cell development does not include the steps involved in committing an unspecified cell to the muscle cell fate.
myelination The process in which myelin sheaths are formed and maintained around neurons. Oligodendrocytes in the brain and spinal cord and Schwann cells in the peripheral nervous system wrap axons with compact layers of their plasma membrane. Adjacent myelin segments are separated by a non-myelinated stretch of axon called a node of Ranvier.
nerve development The process whose specific outcome is the progression of a nerve over time, from its formation to the mature structure.
neuromuscular junction development A process that is carried out at the cellular level which results in the assembly, arrangement of constituent parts, or disassembly of a neuromuscular junction.
protein autoprocessing Processing which a protein carries out itself. This involves actions such as the autolytic removal of residues to generate the mature form of the protein.
protein processing Any protein maturation process achieved by the cleavage of a peptide bond or bonds within a protein. Protein maturation is the process leading to the attainment of the full functional capacity of a protein.
protein-containing complex assembly The aggregation, arrangement and bonding together of a set of macromolecules to form a protein-containing complex.
proteolysis The hydrolysis of proteins into smaller polypeptides and/or amino acids by cleavage of their peptide bonds.
regulation of multicellular organism growth Any process that modulates the frequency, rate or extent of growth of the body of an organism so that it reaches its usual body size.
righting reflex A reflex process in which an animal immediately tries to turn over after being placed in a supine position.

5 homologous proteins in AiPD

UniProt AC Gene Name Protein Name Species Evidence Code
Q9UQ90 SPG7 Paraplegin Homo sapiens (Human) PR
Q9Y4W6 AFG3L2 AFG3-like protein 2 Homo sapiens (Human) PR
Q3ULF4 Spg7 Paraplegin Mus musculus (Mouse) PR
Q7TT47 Spg7 Paraplegin Rattus norvegicus (Rat) PR
Q8W585 FTSH8 ATP-dependent zinc metalloprotease FTSH 8, chloroplastic Arabidopsis thaliana (Mouse-ear cress) PR
10 20 30 40 50 60
MAHRCLLLWG RGACRPRGMP PMLLPGGRTG STERLYLRML YRYATTQAKT SRNSLLTDVI
70 80 90 100 110 120
AAYQRLCSRP PKGFEKYFPN GKNGKKTSEP KEVMGEKKEP KPAAAPRPSG GGVGGGGKRG
130 140 150 160 170 180
GKKDDSHWWS RFQKGDIPWD DKEFKMYFLW TALFWGGFLF YFLFKSSGRE ITWKDFANNY
190 200 210 220 230 240
LSKGVVDRLE VVNKRFVRVT FTPGKTPVDG QYVWFNIGSV DTFERNLETL QQELGIEGEN
250 260 270 280 290 300
RVPVVYIAES DGSFLLSMLP TVLIIAFLLY TIRRGPAGIG RTGRGMGGLF SVGETTAKVL
310 320 330 340 350 360
KDEIDVKFKD VAGCEEAKLE IMEFVNFLKN PKQYQDLGAK IPKGAILTGP PGTGKTLLAK
370 380 390 400 410 420
ATAGEANVPF ITVSGSEFLE MFVGVGPARV RDLFALARKN APCILFIDEI DAVGRKRGRG
430 440 450 460 470 480
NFGGQSEQEN TLNQLLVEMD GFNTTTNVVI LAGTNRPDIL DPALMRPGRF DRQIFIGPPD
490 500 510 520 530 540
IKGRASIFKV HLRPLKLDST LEKEKLARKL ASLTPGFSGA DVANVCNEAA LIAARHLSDS
550 560 570 580 590 600
INQKHFEQAI ERVIGGLEKK TQVLQPEEKK TVAYHEAGHA VAGWYLEHAD PLLKVSIIPR
610 620 630 640 650 660
GKGLGYAQYL PREQYLYTRE QLLDRMCMTL GGRVSEEIFF GRITTGAQDD LRKVTQSAYA
670 680 690 700 710 720
QIVQFGMNEK VGQISFDLPR QGDMVLEKPY SEATARLIDD EVRILINDAY KRTVALLTEK
730 740 750 760 770 780
KADVEKVALL LLEKEVLDKN DMVELLGPRP FAEKSTYEEF VEGTGSLDED TSLPEGLKDW
790 800
NREREGSEEP SGEKVTSPVQ GAGPA