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Protein

AFG3-like protein 2

Gene

Afg3l2

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: 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

GO - Biological processi

  • axonogenesis Source: MGI
  • cristae formation Source: MGI
  • mitochondrial fusion Source: MGI
  • mitochondrial protein processing Source: MGI
  • mitochondrion organization Source: MGI
  • muscle fiber development Source: MGI
  • myelination Source: MGI
  • nerve development Source: MGI
  • neuromuscular junction development Source: MGI
  • regulation of multicellular organism growth Source: MGI
  • righting reflex Source: MGI
Complete GO annotation...

Keywords - Molecular functioni

Hydrolase, Metalloprotease, Protease

Keywords - Ligandi

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

Enzyme and pathway databases

BRENDAi3.4.24.B18. 3474.

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 Componenti: 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

  • integral component of membrane Source: UniProtKB-KW
  • mitochondrial inner membrane Source: MGI
  • 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.
GenevisibleiQ8JZQ2. MM.

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.
GeneTreeiENSGT00800000124136.
HOGENOMiHOG000217277.
HOVERGENiHBG050184.
InParanoidiQ8JZQ2.
KOiK08956.
OMAiTKMDKNA.
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.
CCDSiCCDS37847.1.
RefSeqiNP_081406.1. NM_027130.1.
UniGeneiMm.426052.

3D structure databases

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

Protein-protein interaction databases

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

Protein family/group databases

MEROPSiM41.007.

PTM databases

PhosphoSiteiQ8JZQ2.

Proteomic databases

MaxQBiQ8JZQ2.
PaxDbiQ8JZQ2.
PRIDEiQ8JZQ2.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi10939.
MGIiMGI:1916847. Afg3l2.

Phylogenomic databases

eggNOGiCOG0465.
GeneTreeiENSGT00800000124136.
HOGENOMiHOG000217277.
HOVERGENiHBG050184.
InParanoidiQ8JZQ2.
KOiK08956.
OMAiTKMDKNA.
OrthoDBiEOG7NW68F.
PhylomeDBiQ8JZQ2.
TreeFamiTF105004.

Enzyme and pathway databases

BRENDAi3.4.24.B18. 3474.

Miscellaneous databases

NextBioi329880.
PROiQ8JZQ2.
SOURCEiSearch...

Gene expression databases

BgeeiQ8JZQ2.
CleanExiMM_AFG3L2.
ExpressionAtlasiQ8JZQ2. baseline and differential.
GenevisibleiQ8JZQ2. MM.

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]
ProtoNetiSearch...

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 22, 2015
This is version 111 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

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.