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Q99K67

- AASS_MOUSE

UniProt

Q99K67 - AASS_MOUSE

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Protein

Alpha-aminoadipic semialdehyde synthase, mitochondrial

Gene

Aass

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

Functioni

Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-oxoglutarate reductase and saccharopine dehydrogenase activity, respectively.

Catalytic activityi

N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NADP+ + H2O = L-lysine + 2-oxoglutarate + NADPH.
N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NAD+ + H2O = L-glutamate + (S)-2-amino-6-oxohexanoate + NADH.

Pathwayi

GO - Molecular functioni

  1. saccharopine dehydrogenase (NAD+, L-glutamate-forming) activity Source: UniProtKB
  2. saccharopine dehydrogenase (NADP+, L-lysine-forming) activity Source: UniProtKB-EC

GO - Biological processi

  1. generation of precursor metabolites and energy Source: UniProtKB
  2. L-lysine catabolic process Source: MGI
  3. L-lysine catabolic process to acetyl-CoA via saccharopine Source: UniProtKB-UniPathway
Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Ligandi

NAD, NADP

Enzyme and pathway databases

UniPathwayiUPA00868; UER00835.
UPA00868; UER00836.

Names & Taxonomyi

Protein namesi
Recommended name:
Alpha-aminoadipic semialdehyde synthase, mitochondrial
Alternative name(s):
LKR/SDH
Including the following 2 domains:
Lysine ketoglutarate reductase (EC:1.5.1.8)
Short name:
LKR
Short name:
LOR
Saccharopine dehydrogenase (EC:1.5.1.9)
Short name:
SDH
Gene namesi
Name:Aass
Synonyms:Lorsdh
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
ProteomesiUP000000589: Chromosome 6

Organism-specific databases

MGIiMGI:1353573. Aass.

Subcellular locationi

GO - Cellular componenti

  1. mitochondrion Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3232MitochondrionAdd
BLAST
Chaini33 – 926894Alpha-aminoadipic semialdehyde synthase, mitochondrialPRO_0000001053Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei48 – 481N6-acetyllysine1 Publication
Modified residuei52 – 521N6-acetyllysine1 Publication
Modified residuei56 – 561N6-acetyllysine1 Publication
Modified residuei93 – 931N6-acetyllysine; alternate1 Publication
Modified residuei93 – 931N6-succinyllysine; alternate1 Publication
Modified residuei128 – 1281N6-acetyllysine1 Publication
Modified residuei138 – 1381N6-acetyllysine; alternate1 Publication
Modified residuei138 – 1381N6-succinyllysine; alternate1 Publication
Modified residuei274 – 2741N6-succinyllysine1 Publication
Modified residuei286 – 2861N6-acetyllysine; alternate1 Publication
Modified residuei286 – 2861N6-succinyllysine; alternate1 Publication
Modified residuei333 – 3331N6-succinyllysine1 Publication
Modified residuei458 – 4581N6-acetyllysine; alternate1 Publication
Modified residuei458 – 4581N6-succinyllysine; alternate1 Publication
Modified residuei523 – 5231N6-acetyllysine; alternate1 Publication
Modified residuei523 – 5231N6-succinyllysine; alternate1 Publication
Modified residuei535 – 5351N6-acetyllysine; alternate1 Publication
Modified residuei535 – 5351N6-succinyllysine; alternate1 Publication
Modified residuei584 – 5841N6-acetyllysine; alternate1 Publication
Modified residuei584 – 5841N6-succinyllysine; alternate1 Publication
Modified residuei707 – 7071N6-acetyllysine1 Publication
Modified residuei732 – 7321N6-succinyllysine1 Publication
Modified residuei739 – 7391N6-acetyllysine1 Publication
Modified residuei761 – 7611N6-acetyllysine; alternate1 Publication
Modified residuei761 – 7611N6-succinyllysine; alternate1 Publication
Modified residuei778 – 7781N6-acetyllysine1 Publication
Modified residuei780 – 7801N6-acetyllysine1 Publication

Keywords - PTMi

Acetylation

Proteomic databases

MaxQBiQ99K67.
PaxDbiQ99K67.
PRIDEiQ99K67.

2D gel databases

REPRODUCTION-2DPAGEQ99K67.

PTM databases

PhosphoSiteiQ99K67.

Expressioni

Tissue specificityi

Both enzymes are highly expressed in kidney and liver, very low expression is seen in heart, brain, spleen, lung, skeletal muscle and testis. SDH was detected only in cortical regions of the kidney.

Inductioni

Induced by starvation.

Gene expression databases

BgeeiQ99K67.
CleanExiMM_AASS.
ExpressionAtlasiQ99K67. baseline and differential.
GenevestigatoriQ99K67.

Interactioni

Subunit structurei

Homodimer.

Protein-protein interaction databases

IntActiQ99K67. 2 interactions.
MINTiMINT-1840773.

Structurei

3D structure databases

ProteinModelPortaliQ99K67.
SMRiQ99K67. Positions 30-424, 481-912.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni33 – 455423Lysine-ketoglutarate reductaseAdd
BLAST
Regioni477 – 926450Saccharopine dehydrogenaseAdd
BLAST

Sequence similaritiesi

In the N-terminal section; belongs to the AlaDH/PNT family.Curated
In the C-terminal section; belongs to the saccharopine dehydrogenase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG1748.
GeneTreeiENSGT00390000013249.
HOGENOMiHOG000252920.
HOVERGENiHBG048688.
InParanoidiQ99K67.
KOiK14157.
OMAiILKQEIR.
OrthoDBiEOG7F7W7Z.
PhylomeDBiQ99K67.
TreeFamiTF105728.

Family and domain databases

Gene3Di3.40.50.720. 1 hit.
InterProiIPR007886. AlaDH/PNT_N.
IPR007698. AlaDH/PNT_NAD(H)-bd.
IPR016040. NAD(P)-bd_dom.
IPR005097. Saccharopine_DH/HSpermid_syn.
[Graphical view]
PfamiPF01262. AlaDh_PNT_C. 1 hit.
PF05222. AlaDh_PNT_N. 1 hit.
PF03435. Saccharop_dh. 1 hit.
[Graphical view]
SMARTiSM01002. AlaDh_PNT_C. 1 hit.
SM01003. AlaDh_PNT_N. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

Q99K67-1 [UniParc]FASTAAdd to Basket

« Hide

        10         20         30         40         50
MLRAQRPRLA RLRACLSRGL HHKPVMALRR EDVNAWERRA PLAPKHIKGI
60 70 80 90 100
TKLGYKVLIQ PSNRRAIHDK EYVRAGGILQ EDITEACLIL GVKRPPEEKL
110 120 130 140 150
MSKKTYAFFS HTIKAQEANM NLLDEVLKQE IRLIDYEKMV DHRGSRIVAF
160 170 180 190 200
GQWAGVAGMI NILHGMGLRL LALGHHTPFM HLGMAHNYRN SSQAVQAVRD
210 220 230 240 250
AGYEISLGLM PKSIGPLTFV FTGTGNVSKG AQEVFNELPC EYVEPHELRE
260 270 280 290 300
VSKTGDLRKV YGTVLSRHHH LVRKTDGVYD PVEYEKYPER YTSRFNTDIA
310 320 330 340 350
PYTTCLINGI YWEQNTPRLL TRQDAQSLLV PVKSSVVPVE GCPELPHKLV
360 370 380 390 400
AICDISADTG GSIDFMTECT TIERPFCMYD ADQQIIHDSV EGSGILMCSI
410 420 430 440 450
DNLPAQLPIE ATEYFGDMLY PYVEEMLLSD ASQPLESQNF SPVVRDAVIT
460 470 480 490 500
SNGLLTDKYK YIQKLRESRE RIQFLSMSTK KKVLVLGSGY VSGPVLEYLS
510 520 530 540 550
RDNNIEITLG SDMTNQMQQL SKKYNINPVS LTVGKQEAKL QSLVESQDLV
560 570 580 590 600
ISLLPYVLHP VVAKACIESR VNMVTASYIT PAMKELEKSV DDAGITVIGE
610 620 630 640 650
LGLDPGLDHM LAMETIDTAK ELGATVESYV SYCGGLPAPE HSDNPLRYKF
660 670 680 690 700
SWSPVGVLMN IMQPASYLLN GKVVNVTGGV SFLNSVTPMD YFPGLNLEGY
710 720 730 740 750
PNRDSIKYAE IYGISSAHTL LRGTLRYKGY SKALNGFVKL GLINREAYPA
760 770 780 790 800
LRPEANPLTW KQLLCDLVGI SRSSPCEKLK EVVFTKLGGD NTQLEAAEWL
810 820 830 840 850
GLLGDEQVPQ AESIVDAFSK HLVSKLSYGP EEKDMIVMRD SFGIRHPSGH
860 870 880 890 900
LENKTIDLVV YGDFNGFSAM AKTVGLPTAM AAKMLLDGEI EAKGLMGPFT
910 920
KEIYGPILER IKAEGIVFNT QSTIKL
Length:926
Mass (Da):102,975
Last modified:June 1, 2001 - v1
Checksum:i5B4369C51F7D1D53
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti198 – 1981V → I in CAA12114. (PubMed:10567240)Curated
Sequence conflicti851 – 8511L → A in CAA12114. (PubMed:10567240)Curated

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
AJ224761 mRNA. Translation: CAA12114.1.
BC005420 mRNA. Translation: AAH05420.1.
CCDSiCCDS19937.1.
RefSeqiNP_038958.2. NM_013930.4.
UniGeneiMm.18651.

Genome annotation databases

EnsembliENSMUST00000031707; ENSMUSP00000031707; ENSMUSG00000029695.
GeneIDi30956.
KEGGimmu:30956.
UCSCiuc009bbc.2. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
AJ224761 mRNA. Translation: CAA12114.1 .
BC005420 mRNA. Translation: AAH05420.1 .
CCDSi CCDS19937.1.
RefSeqi NP_038958.2. NM_013930.4.
UniGenei Mm.18651.

3D structure databases

ProteinModelPortali Q99K67.
SMRi Q99K67. Positions 30-424, 481-912.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

IntActi Q99K67. 2 interactions.
MINTi MINT-1840773.

PTM databases

PhosphoSitei Q99K67.

2D gel databases

REPRODUCTION-2DPAGE Q99K67.

Proteomic databases

MaxQBi Q99K67.
PaxDbi Q99K67.
PRIDEi Q99K67.

Protocols and materials databases

Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENSMUST00000031707 ; ENSMUSP00000031707 ; ENSMUSG00000029695 .
GeneIDi 30956.
KEGGi mmu:30956.
UCSCi uc009bbc.2. mouse.

Organism-specific databases

CTDi 10157.
MGIi MGI:1353573. Aass.

Phylogenomic databases

eggNOGi COG1748.
GeneTreei ENSGT00390000013249.
HOGENOMi HOG000252920.
HOVERGENi HBG048688.
InParanoidi Q99K67.
KOi K14157.
OMAi ILKQEIR.
OrthoDBi EOG7F7W7Z.
PhylomeDBi Q99K67.
TreeFami TF105728.

Enzyme and pathway databases

UniPathwayi UPA00868 ; UER00835 .
UPA00868 ; UER00836 .

Miscellaneous databases

NextBioi 307440.
PROi Q99K67.
SOURCEi Search...

Gene expression databases

Bgeei Q99K67.
CleanExi MM_AASS.
ExpressionAtlasi Q99K67. baseline and differential.
Genevestigatori Q99K67.

Family and domain databases

Gene3Di 3.40.50.720. 1 hit.
InterProi IPR007886. AlaDH/PNT_N.
IPR007698. AlaDH/PNT_NAD(H)-bd.
IPR016040. NAD(P)-bd_dom.
IPR005097. Saccharopine_DH/HSpermid_syn.
[Graphical view ]
Pfami PF01262. AlaDh_PNT_C. 1 hit.
PF05222. AlaDh_PNT_N. 1 hit.
PF03435. Saccharop_dh. 1 hit.
[Graphical view ]
SMARTi SM01002. AlaDh_PNT_C. 1 hit.
SM01003. AlaDh_PNT_N. 1 hit.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "Lysine degradation through the saccharopine pathway in mammals: involvement of both bifunctional and monofunctional lysine-degrading enzymes in mouse."
    Papes F., Kemper E.L., Cord-Neto G., Langone F., Arruda P.
    Biochem. J. 344:555-563(1999) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    Strain: C57BL/6J X CBA/J.
    Tissue: Liver.
  2. "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].
    Tissue: Mammary tumor.
  3. "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-93; LYS-138; LYS-274; LYS-286; LYS-333; LYS-458; LYS-523; LYS-535; LYS-584; LYS-732 AND LYS-761, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.
  4. "Label-free quantitative proteomics of the lysine acetylome in mitochondria identifies substrates of SIRT3 in metabolic pathways."
    Rardin M.J., Newman J.C., Held J.M., Cusack M.P., Sorensen D.J., Li B., Schilling B., Mooney S.D., Kahn C.R., Verdin E., Gibson B.W.
    Proc. Natl. Acad. Sci. U.S.A. 110:6601-6606(2013) [PubMed] [Europe PMC] [Abstract]
    Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-48; LYS-52; LYS-56; LYS-93; LYS-128; LYS-138; LYS-286; LYS-458; LYS-523; LYS-535; LYS-584; LYS-707; LYS-739; LYS-761; LYS-778 AND LYS-780, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.

Entry informationi

Entry nameiAASS_MOUSE
AccessioniPrimary (citable) accession number: Q99K67
Secondary accession number(s): Q9Z1I9
Entry historyi
Integrated into UniProtKB/Swiss-Prot: April 13, 2004
Last sequence update: June 1, 2001
Last modified: October 29, 2014
This is version 107 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, Multifunctional enzyme, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3