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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

Binds 1 zinc ion per subunit Reviewed prediction.

Sites

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

Regions

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Nucleotide bindingi347 – 3548ATP Reviewed prediction

GO - Molecular functioni

  1. ATP binding Source: UniProtKB-KW
  2. metalloendopeptidase activity Source: InterPro
  3. nucleoside-triphosphatase activity Source: InterPro
  4. 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 – 16221Helical; Reviewed predictionAdd
BLAST
Transmembranei250 – 27021Helical; Reviewed predictionAdd
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 2UniRule annotationPRO_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

ArrayExpressiQ8JZQ2.
BgeeiQ8JZQ2.
CleanExiMM_AFG3L2.
GenevestigatoriQ8JZQ2.

Interactioni

Subunit structurei

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

Protein-protein interaction databases

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

Structurei

3D structure databases

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

Family & Domainsi

Sequence similaritiesi

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

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

MAHRCLLLWS RGGCRRGLPP LLVPRGCLGP DRRPCLRTLY QYATVQTASS    50
RRSLLRDVIA AYQRFCSRPP KGFEKYFPNG KNGKKASEPK EAVGEKKEPQ 100
PSGPQPSGGA GGGGGKRRGK KEDSHWWSRF QKGDFPWDDK DFRMYFLWTA 150
LFWGGVMIYF VFKSSGREIT WKDFVNNYLS KGVVDRLEVV NKRFVRVTFT 200
PGKTPVDGQY VWFNIGSVDT FERNLETLQQ ELGIEGENRV PVVYIAESDG 250
SFLLSMLPTV LIIAFLLYTI RRGPAGIGRT GRGMGGLFSV GETTAKVLKD 300
EIDVKFKDVA GCEEAKLEIM EFVNFLKNPK QYQDLGAKIP KGAILTGPPG 350
TGKTLLAKAT AGEANVPFIT VSGSEFLEMF VGVGPARVRD LFALARKNAP 400
CILFIDEIDA VGRKRGRGNF GGQSEQENTL NQLLVEMDGF NTTTNVVILA 450
GTNRPDILDP ALLRPGRFDR QIFIGPPDIK GRASIFKVHL RPLKLDSALE 500
KDKLARKLAS LTPGFSGADV ANVCNEAALI AARHLSDAIN EKHFEQAIER 550
VIGGLEKKTQ VLQPEEKKTV AYHEAGHAVA GWYLEHADPL LKVSIIPRGK 600
GLGYAQYLPK EQYLYTKEQL LDRMCMTLGG RVSEEIFFGR ITTGAQDDLR 650
KVTQSAYAQI VQFGMNEKVG QISFDLPRQG DMVLEKPYSE ATARMIDDEV 700
RILISDAYRR TVALLTEKKA DVEKVALLLL EKEVLDKNDM VQLLGPRPFT 750
EKSTYEEFVE GTGSLDEDTS LPEGLQDWNK EREKEEKKEK EKEEPLNEKV 800
VS 802
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:
EMBL
GenBank
DDBJ
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:
EMBL
GenBank
DDBJ
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

ArrayExpressi Q8JZQ2.
Bgeei Q8JZQ2.
CleanExi MM_AFG3L2.
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: July 9, 2014
This is version 103 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

Similar proteinsi