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Q8JZQ2

- AFG32_MOUSE

UniProt

Q8JZQ2 - AFG32_MOUSE

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Protein

AFG3-like protein 2

Gene

Afg3l2

Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli

Functioni

ATP-dependent protease which is essential for axonal development.1 Publication

Cofactori

Zn2+CuratedNote: Binds 1 zinc ion per subunit.Curated

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi573 – 5731Zinc; catalyticBy similarity
Active sitei574 – 5741By similarity
Metal bindingi577 – 5771Zinc; catalyticBy similarity
Metal bindingi648 – 6481Zinc; catalyticBy similarity

Regions

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Nucleotide bindingi347 – 3548ATPSequence Analysis

GO - Molecular functioni

  1. ATP binding Source: UniProtKB-KW
  2. metalloendopeptidase activity Source: InterPro
  3. zinc ion binding Source: InterPro

GO - Biological processi

  1. axonogenesis Source: MGI
  2. cristae formation Source: MGI
  3. death Source: MGI
  4. mitochondrial fusion Source: MGI
  5. mitochondrial protein processing Source: MGI
  6. mitochondrion organization Source: MGI
  7. muscle fiber development Source: MGI
  8. myelination Source: MGI
  9. nerve development Source: MGI
  10. neuromuscular junction development Source: MGI
  11. regulation of multicellular organism growth Source: MGI
  12. righting reflex Source: MGI
Complete GO annotation...

Keywords - Molecular functioni

Hydrolase, Metalloprotease, Protease

Keywords - Ligandi

ATP-binding, Metal-binding, Nucleotide-binding, Zinc

Protein family/group databases

MEROPSiM41.007.

Names & Taxonomyi

Protein namesi
Recommended name:
AFG3-like protein 2 (EC:3.4.24.-)
Gene namesi
Name:Afg3l2
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
ProteomesiUP000000589: Chromosome 18

Organism-specific databases

MGIiMGI:1916847. Afg3l2.

Subcellular locationi

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transmembranei142 – 16221HelicalSequence AnalysisAdd
BLAST
Transmembranei250 – 27021HelicalSequence AnalysisAdd
BLAST

GO - Cellular componenti

  1. integral component of membrane Source: UniProtKB-KW
  2. mitochondrial inner membrane Source: MGI
  3. mitochondrion Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion

Pathology & Biotechi

Involvement in diseasei

Defects in Afg3l2 are the cause of the paralyze (par) phenotype, a spontaneous mutant strain. Par mice have a normal appearance and fertility but are significantly smaller than their littermates at 1 week of age and display a rapidly progressive loss of motor function in all limbs by 12-14 days. As the disease progresses, they lose the ability to support their own weight or turn themselves over when placed on their back and exhibit a typical posture with over extension of all limbs and uncoordinated movements. They rarely survive beyond 16 days of age, when they are completely paralyzed.

Keywords - Diseasei

Disease mutation

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 802802AFG3-like protein 2PRO_0000084674Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei116 – 1161N6-succinyllysine1 Publication

Proteomic databases

MaxQBiQ8JZQ2.
PaxDbiQ8JZQ2.
PRIDEiQ8JZQ2.

PTM databases

PhosphoSiteiQ8JZQ2.

Expressioni

Tissue specificityi

Highly expressed in the cerebellar Purkinje cells.1 Publication

Gene expression databases

BgeeiQ8JZQ2.
CleanExiMM_AFG3L2.
ExpressionAtlasiQ8JZQ2. baseline and differential.
GenevestigatoriQ8JZQ2.

Interactioni

Subunit structurei

Homooligomer. Interacts with SPG7; the interaction is required for the efficient assembly of mitochondrial complex I (By similarity).By similarity

Protein-protein interaction databases

IntActiQ8JZQ2. 3 interactions.
MINTiMINT-4116623.
STRINGi10090.ENSMUSP00000025408.

Structurei

3D structure databases

ProteinModelPortaliQ8JZQ2.
SMRiQ8JZQ2. Positions 163-250, 295-749.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

In the N-terminal section; belongs to the AAA ATPase family.Curated
In the C-terminal section; belongs to the peptidase M41 family.Curated

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiCOG0465.
GeneTreeiENSGT00730000110911.
HOGENOMiHOG000217277.
HOVERGENiHBG050184.
InParanoidiQ8JZQ2.
KOiK08956.
OMAiMAHRCLR.
OrthoDBiEOG7NW68F.
PhylomeDBiQ8JZQ2.
TreeFamiTF105004.

Family and domain databases

Gene3Di3.40.50.300. 1 hit.
HAMAPiMF_01458. FtsH.
InterProiIPR003593. AAA+_ATPase.
IPR003959. ATPase_AAA_core.
IPR003960. ATPase_AAA_CS.
IPR005936. FtsH.
IPR027417. P-loop_NTPase.
IPR011546. Pept_M41_FtsH_extracell.
IPR000642. Peptidase_M41.
[Graphical view]
PfamiPF00004. AAA. 1 hit.
PF06480. FtsH_ext. 1 hit.
PF01434. Peptidase_M41. 1 hit.
[Graphical view]
SMARTiSM00382. AAA. 1 hit.
[Graphical view]
SUPFAMiSSF52540. SSF52540. 1 hit.
TIGRFAMsiTIGR01241. FtsH_fam. 1 hit.
PROSITEiPS00674. AAA. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Q8JZQ2-1 [UniParc]FASTAAdd to Basket

« Hide

        10         20         30         40         50
MAHRCLLLWS RGGCRRGLPP LLVPRGCLGP DRRPCLRTLY QYATVQTASS
60 70 80 90 100
RRSLLRDVIA AYQRFCSRPP KGFEKYFPNG KNGKKASEPK EAVGEKKEPQ
110 120 130 140 150
PSGPQPSGGA GGGGGKRRGK KEDSHWWSRF QKGDFPWDDK DFRMYFLWTA
160 170 180 190 200
LFWGGVMIYF VFKSSGREIT WKDFVNNYLS KGVVDRLEVV NKRFVRVTFT
210 220 230 240 250
PGKTPVDGQY VWFNIGSVDT FERNLETLQQ ELGIEGENRV PVVYIAESDG
260 270 280 290 300
SFLLSMLPTV LIIAFLLYTI RRGPAGIGRT GRGMGGLFSV GETTAKVLKD
310 320 330 340 350
EIDVKFKDVA GCEEAKLEIM EFVNFLKNPK QYQDLGAKIP KGAILTGPPG
360 370 380 390 400
TGKTLLAKAT AGEANVPFIT VSGSEFLEMF VGVGPARVRD LFALARKNAP
410 420 430 440 450
CILFIDEIDA VGRKRGRGNF GGQSEQENTL NQLLVEMDGF NTTTNVVILA
460 470 480 490 500
GTNRPDILDP ALLRPGRFDR QIFIGPPDIK GRASIFKVHL RPLKLDSALE
510 520 530 540 550
KDKLARKLAS LTPGFSGADV ANVCNEAALI AARHLSDAIN EKHFEQAIER
560 570 580 590 600
VIGGLEKKTQ VLQPEEKKTV AYHEAGHAVA GWYLEHADPL LKVSIIPRGK
610 620 630 640 650
GLGYAQYLPK EQYLYTKEQL LDRMCMTLGG RVSEEIFFGR ITTGAQDDLR
660 670 680 690 700
KVTQSAYAQI VQFGMNEKVG QISFDLPRQG DMVLEKPYSE ATARMIDDEV
710 720 730 740 750
RILISDAYRR TVALLTEKKA DVEKVALLLL EKEVLDKNDM VQLLGPRPFT
760 770 780 790 800
EKSTYEEFVE GTGSLDEDTS LPEGLQDWNK EREKEEKKEK EKEEPLNEKV

VS
Length:802
Mass (Da):89,519
Last modified:October 1, 2002 - v1
Checksum:iE7300BD686532D2D
GO

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti389 – 3891R → G in par. 1 Publication

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
BC036999 mRNA. Translation: AAH36999.1.
BC043056 mRNA. Translation: AAH43056.1.
CCDSiCCDS37847.1.
RefSeqiNP_081406.1. NM_027130.1.
UniGeneiMm.426052.

Genome annotation databases

EnsembliENSMUST00000025408; ENSMUSP00000025408; ENSMUSG00000024527.
GeneIDi69597.
KEGGimmu:69597.
UCSCiuc008fmf.1. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
BC036999 mRNA. Translation: AAH36999.1 .
BC043056 mRNA. Translation: AAH43056.1 .
CCDSi CCDS37847.1.
RefSeqi NP_081406.1. NM_027130.1.
UniGenei Mm.426052.

3D structure databases

ProteinModelPortali Q8JZQ2.
SMRi Q8JZQ2. Positions 163-250, 295-749.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

IntActi Q8JZQ2. 3 interactions.
MINTi MINT-4116623.
STRINGi 10090.ENSMUSP00000025408.

Protein family/group databases

MEROPSi M41.007.

PTM databases

PhosphoSitei Q8JZQ2.

Proteomic databases

MaxQBi Q8JZQ2.
PaxDbi Q8JZQ2.
PRIDEi Q8JZQ2.

Protocols and materials databases

Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENSMUST00000025408 ; ENSMUSP00000025408 ; ENSMUSG00000024527 .
GeneIDi 69597.
KEGGi mmu:69597.
UCSCi uc008fmf.1. mouse.

Organism-specific databases

CTDi 10939.
MGIi MGI:1916847. Afg3l2.

Phylogenomic databases

eggNOGi COG0465.
GeneTreei ENSGT00730000110911.
HOGENOMi HOG000217277.
HOVERGENi HBG050184.
InParanoidi Q8JZQ2.
KOi K08956.
OMAi MAHRCLR.
OrthoDBi EOG7NW68F.
PhylomeDBi Q8JZQ2.
TreeFami TF105004.

Miscellaneous databases

NextBioi 329880.
PROi Q8JZQ2.
SOURCEi Search...

Gene expression databases

Bgeei Q8JZQ2.
CleanExi MM_AFG3L2.
ExpressionAtlasi Q8JZQ2. baseline and differential.
Genevestigatori Q8JZQ2.

Family and domain databases

Gene3Di 3.40.50.300. 1 hit.
HAMAPi MF_01458. FtsH.
InterProi IPR003593. AAA+_ATPase.
IPR003959. ATPase_AAA_core.
IPR003960. ATPase_AAA_CS.
IPR005936. FtsH.
IPR027417. P-loop_NTPase.
IPR011546. Pept_M41_FtsH_extracell.
IPR000642. Peptidase_M41.
[Graphical view ]
Pfami PF00004. AAA. 1 hit.
PF06480. FtsH_ext. 1 hit.
PF01434. Peptidase_M41. 1 hit.
[Graphical view ]
SMARTi SM00382. AAA. 1 hit.
[Graphical view ]
SUPFAMi SSF52540. SSF52540. 1 hit.
TIGRFAMsi TIGR01241. FtsH_fam. 1 hit.
PROSITEi PS00674. AAA. 1 hit.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Strain: C57BL/6 and FVB/N.
    Tissue: Brain and Kidney.
  2. Lubec G., Kang S.U.
    Submitted (APR-2007) to UniProtKB
    Cited for: PROTEIN SEQUENCE OF 173-181; 283-296 AND 632-650, IDENTIFICATION BY MASS SPECTROMETRY.
    Strain: C57BL/6.
    Tissue: Brain.
  3. Cited for: FUNCTION, VARIANT PAR GLY-389.
  4. Cited for: TISSUE SPECIFICITY.
  5. "SIRT5-mediated lysine desuccinylation impacts diverse metabolic pathways."
    Park J., Chen Y., Tishkoff D.X., Peng C., Tan M., Dai L., Xie Z., Zhang Y., Zwaans B.M., Skinner M.E., Lombard D.B., Zhao Y.
    Mol. Cell 50:919-930(2013) [PubMed] [Europe PMC] [Abstract]
    Cited for: SUCCINYLATION [LARGE SCALE ANALYSIS] AT LYS-116, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.

Entry informationi

Entry nameiAFG32_MOUSE
AccessioniPrimary (citable) accession number: Q8JZQ2
Entry historyi
Integrated into UniProtKB/Swiss-Prot: September 13, 2005
Last sequence update: October 1, 2002
Last modified: November 26, 2014
This is version 106 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. Peptidase families
    Classification of peptidase families and list of entries
  3. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3