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Gene Model: Sucla2

NomenclatureGenomic Location
SymbolSucla2Chromosome1
NameSuccinate-CoA ligase, ADP-forming,
beta subunit		Linkage mapunknown
SpeciesDracomimus familiarisGenome Coordinates1: 72 Mbp

Molecular Function

ATP + succinate + CoA = ADP + phosphate + succinyl-CoA.

Molecular Function Terms:

binding
   ion binding
      cation binding
         metal ion binding
   nucleotide binding
      purine nucleotide binding
         adenyl nucleotide binding
            adenyl ribonucleotide binding
               ATP binding

catalytic activity
   ligase activity
      ligase activity, forming carbon-sulfur bonds
         acid-thiol ligase activity
            succinate-CoA ligase activity

Human Disease Association

Defects in SUCLA2 are the cause of mitochondrial DNA depletion syndrome type 5 (MTDPS5) [MIM:612073]. A disorder characterized by infantile onset of hypotonia, neurologic deterioration, a hyperkinetic-dystonic movement disorder, external ophthalmoplegia, deafness, variable renal tubular dysfunction, and mild methylmalonic aciduria.

Predicted Transcript
     1 ATGGCCGCCTCCATGATGTGTTCGCGGGTGGTGACAGCGGCCGCCAGGCAGCTGAGGGGA
    61 TATCAAGGAGGGCGCGCGAGCCTCGCGGCCAAGATTCTCAGTGGAAGTCCTGGTTTGCTT
   121 AGCAATCATGCATTTCAAATTGAGCAGCGAAGGAACTTGTCATTGCATGAATATCTGAGT
   181 ATGGAACTACTGAAAGAAGCTGGAATTTCTGTTCCACATGGATTAGTTGCAAATACACCA
   241 GAGGACGCTTATAACGTTGCCAAACAAATAGGTACAAAAGACCTTGTTGTGAAGGCACAA
   301 GTTTTAGCTGGTGGCAGAGGAAAAGGAACATTTGAAGGTGGTCTAAAAGGAGGAGTGAAA
   361 ATAGTCTTTTCTCCAGAAGAAGCACGAGAGATTTCCTCAAGAATGATTGGGAAAAAGCTA
   421 TTCACAAAGCAGACGGGAGAAAAGGGCAGGATATGCAATCAAGTATTTATCTGTGAGCGC
   481 AGATATCCCAGAAGAGAGTACTATTTTGCAATAACCATGGAACGATCATTTCAGGGCCCT
   541 GTACTCATTGGCAGCGCTCAAGGTGGTGTCAATATTGAAGATGTGGCTGCAGAGAATCCT
   601 GATGCTATTGCCAAGGAACCAGTTGACATTGTGGAAGGCATCCAGAAGGAACAAGCTATC
   661 AGGCTGGCCAAGAAAATGGGATTTCCTGCTAATTTAGTAAATGAAGCAGCTGAAAATATG
   721 ATTAAGATCTACAACCTCTTTATTAAATTTGATGCCACAATGGTAGAAATTAACCCCATG
   781 GTTGAAGATTCCACAGGGATTGTGATGTGTATGGACGCAAAGATAAACTTTGATAGCAAT
   841 TCAGCTTATCGTCAGCAGAAAATCTTCAGCTTGCAAGACTGGACCCAAGAAGATGACAGA
   901 GACAGGGATGCAGCAAAGTCTGATCTTAATTATATAGGATTAGATGGCAGCATTGGATGT
   961 CTGGTAAATGGTGCTGGTCTTGCAATGGCCACCATGGATATAATTAAACTTCATGGAGGC
  1021 ACTCCAGCAAATTTTCTTGATGTCGGTGGTGGTGCTACAGCGCAGCGGGTGACTGAGGCC
  1081 TTTAGACTTATTACTTCAGATAAAAAGGTGCAGGCAATTTTGGTAAATATTTTTGGAGGC
  1141 ATTATGCGGTGTGATGTGATTGCACAAGGTATAATCATGGCAGTAAAAGATTTGGAACTA
  1201 AAAATACCTATTGTTGTACGGTTACAAGGTACACGAGTTGATGATGCCAAAGCTTTAATA
  1261 ACAGACAGTGGTCTGAAAATACTTGCTTGTGATGATTTGGATGAAGCTGCTAAAATGGTT
  1321 GTGAAACTCTCTGAAATAGTGACCTTAGCAAAGCAGGCGCAAGTGGATGTTAAGTTTCAG
  1381 TTACCAATCTGA

Predicted Protein Product
MAASMMCSRVVTAAARQLRGYQGGRASLAAKILSGSPGLLSNHAFQIEQRRNLSLHEYLS
MELLKEAGISVPHGLVANTPEDAYNVAKQIGTKDLVVKAQVLAGGRGKGTFEGGLKGGVK
IVFSPEEAREISSRMIGKKLFTKQTGEKGRICNQVFICERRYPRREYYFAITMERSFQGP
VLIGSAQGGVNIEDVAAENPDAIAKEPVDIVEGIQKEQAIRLAKKMGFPANLVNEAAENM
IKIYNLFIKFDATMVEINPMVEDSTGIVMCMDAKINFDSNSAYRQQKIFSLQDWTQEDDR
DRDAAKSDLNYIGLDGSIGCLVNGAGLAMATMDIIKLHGGTPANFLDVGGGATAQRVTEA
FRLITSDKKVQAILVNIFGGIMRCDVIAQGIIMAVKDLELKIPIVVRLQGTRVDDAKALI
TDSGLKILACDDLDEAAKMVVKLSEIVTLAKQAQVDVKFQLPI
Protein Alignment to Mouse
sp|Q9Z2I9|SUCB1_MOUSE Succinyl-CoA ligase [ADP-forming] subunit beta,
            mitochondrial OS=Mus musculus GN=Sucla2 PE=1 SV=2
      MGI:1306775 Sucla2 succinate-Coenzyme A ligase, ADP-forming, beta subunit (Chr 14)
        Length = 463

 Score = 1802 (639.4 bits), Expect = 1.7e-186, P = 1.7e-186
 Identities = 353/465 (75%), Positives = 405/465 (87%)

Query:     1 MAASMMCSRVVTAAARQLRGYQGGRASLAAKILSGSPGLLSNHAFQIEQR--RNLSLHEY 58
             MAASM   R + AAA  LR ++      AA  + G+ GL + H  Q++Q+  R LSLHEY
Sbjct:     1 MAASMFYGRQLAAAA--LRSHRPQTTLRAAAQVLGNSGLFNKHGLQVQQQQQRTLSLHEY 58

Query:    59 LSMELLKEAGISVPHGLVANTPEDAYNVAKQIGTKDLVVKAQVLAXXXXXXXXXXXXXXX 118
             LSMELL+EAG+SVP G VA + ++AY +AK++G+KD+V+KAQVLA               
Sbjct:    59 LSMELLQEAGVSVPKGFVAKSSDEAYAIAKKLGSKDVVIKAQVLAGGRGKGTFTSGLKGG 118

Query:   119 XXIVFSPEEAREISSRMIGKKLFTKQTGEKGRICNQVFICERRYPRREYYFAITMERSFQ 178
               IVFSPEEA+ +SS+MIG+KL TKQTGEKGRICNQV +CER+YPRREYYFAITMERSFQ
Sbjct:   119 VKIVFSPEEAKAVSSQMIGQKLITKQTGEKGRICNQVLVCERKYPRREYYFAITMERSFQ 178

Query:   179 GPVLIGSAQGGVNIEDVAAENPDAIAKEPVDIVEGIQKEQAIRLAKKMGFPANLVNEAAE 238
             GPVLIGSAQGGVNIEDVAAENP+AI KEP+DIVEGI+KEQA+ LA+KMGFP+N+V+ AAE
Sbjct:   179 GPVLIGSAQGGVNIEDVAAENPEAIVKEPIDIVEGIKKEQAVTLAQKMGFPSNIVDSAAE 238

Query:   239 NMIKIYNLFIKFDATMVEINPMVEDSTGIVMCMDAKINFDSNSAYRQQKIFSLQDWTQED 298
             NMIK+YNLF+K+DATMVEINPMVEDS G V+CMDAKINFDSNSAYRQ+KIF LQDW+QED
Sbjct:   239 NMIKLYNLFLKYDATMVEINPMVEDSDGKVLCMDAKINFDSNSAYRQKKIFDLQDWSQED 298

Query:   299 DRDRDAAKSDLNYIGLDGSIGCLVNGAGLAMATMDIIKLHGGTPANFLDVGGGATAQRVT 358
             +RD++AA +D+NYIGLDGSIGCLVNGAGLAMATMDIIKLHGGTPANFLDVGGGAT Q+VT
Sbjct:   299 ERDKEAANADINYIGLDGSIGCLVNGAGLAMATMDIIKLHGGTPANFLDVGGGATVQQVT 358

Query:   359 EAFRLITSDKKVQAILVNIFGGIMRCDVIAQGIIMAVKDLELKIPIVVRLQGTRVDDAKA 418
             EAF+LITSDKKVQAILVNIFGGIMRCDVIAQGI+MAVKDLE++IP+VVRLQGTRVDDAKA
Sbjct:   359 EAFKLITSDKKVQAILVNIFGGIMRCDVIAQGIVMAVKDLEIRIPVVVRLQGTRVDDAKA 418

Query:   419 LITDSGLKILACDDLDEAAKMVVKLSEIVTLAKQAQVDVKFQLPI 463
             LI DSGLKILACDDLDEAAKMVVKLSEIVTLAK+A VDVKFQLPI
Sbjct:   419 LIADSGLKILACDDLDEAAKMVVKLSEIVTLAKEAHVDVKFQLPI 463