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Protein

Alpha-aminoadipic semialdehyde synthase, mitochondrial

Gene

Aass

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: 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: L-lysine degradation via saccharopine pathway

This protein is involved in step 1 and 2 of the subpathway that synthesizes glutaryl-CoA from L-lysine.
Proteins known to be involved in the 6 steps of the subpathway in this organism are:
  1. Alpha-aminoadipic semialdehyde synthase, mitochondrial (Aass)
  2. Alpha-aminoadipic semialdehyde synthase, mitochondrial (Aass)
  3. no protein annotated in this organism
  4. Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial (Aadat)
  5. no protein annotated in this organism
  6. Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial (Dlst)
This subpathway is part of the pathway L-lysine degradation via saccharopine pathway, which is itself part of Amino-acid degradation.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes glutaryl-CoA from L-lysine, the pathway L-lysine degradation via saccharopine pathway and in Amino-acid degradation.

GO - Molecular functioni

GO - Biological processi

Keywords - Molecular functioni

Oxidoreductase

Keywords - Ligandi

NAD, NADP

Enzyme and pathway databases

ReactomeiR-MMU-71064. Lysine catabolism.
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
Proteomesi
  • UP000000589 Componenti: Chromosome 6

Organism-specific databases

MGIiMGI:1353573. Aass.

Subcellular locationi

GO - Cellular componenti

  • intracellular membrane-bounded organelle Source: MGI
  • mitochondrion Source: UniProtKB

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 32MitochondrionAdd BLAST32
ChainiPRO_000000105333 – 926Alpha-aminoadipic semialdehyde synthase, mitochondrialAdd BLAST894

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei48N6-acetyllysineCombined sources1
Modified residuei52N6-acetyllysineCombined sources1
Modified residuei56N6-acetyllysineCombined sources1
Modified residuei93N6-acetyllysine; alternateCombined sources1
Modified residuei93N6-succinyllysine; alternateCombined sources1
Modified residuei128N6-acetyllysineCombined sources1
Modified residuei138N6-acetyllysine; alternateCombined sources1
Modified residuei138N6-succinyllysine; alternateCombined sources1
Modified residuei274N6-succinyllysineCombined sources1
Modified residuei286N6-acetyllysine; alternateCombined sources1
Modified residuei286N6-succinyllysine; alternateCombined sources1
Modified residuei333N6-succinyllysineCombined sources1
Modified residuei458N6-acetyllysine; alternateCombined sources1
Modified residuei458N6-succinyllysine; alternateCombined sources1
Modified residuei523N6-acetyllysine; alternateCombined sources1
Modified residuei523N6-succinyllysine; alternateCombined sources1
Modified residuei535N6-acetyllysine; alternateCombined sources1
Modified residuei535N6-succinyllysine; alternateCombined sources1
Modified residuei584N6-acetyllysine; alternateCombined sources1
Modified residuei584N6-succinyllysine; alternateCombined sources1
Modified residuei707N6-acetyllysineCombined sources1
Modified residuei732N6-succinyllysineCombined sources1
Modified residuei739N6-acetyllysineCombined sources1
Modified residuei761N6-acetyllysine; alternateCombined sources1
Modified residuei761N6-succinyllysine; alternateCombined sources1
Modified residuei778N6-acetyllysineCombined sources1
Modified residuei780N6-acetyllysineCombined sources1

Keywords - PTMi

Acetylation

Proteomic databases

MaxQBiQ99K67.
PaxDbiQ99K67.
PRIDEiQ99K67.

2D gel databases

REPRODUCTION-2DPAGEQ99K67.

PTM databases

iPTMnetiQ99K67.
PhosphoSitePlusiQ99K67.

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

BgeeiENSMUSG00000029695.
CleanExiMM_AASS.
ExpressionAtlasiQ99K67. baseline and differential.
GenevisibleiQ99K67. MM.

Interactioni

Subunit structurei

Homodimer.

Protein-protein interaction databases

IntActiQ99K67. 2 interactors.
MINTiMINT-1840773.
STRINGi10090.ENSMUSP00000031707.

Structurei

3D structure databases

ProteinModelPortaliQ99K67.
SMRiQ99K67.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni33 – 455Lysine-ketoglutarate reductaseAdd BLAST423
Regioni477 – 926Saccharopine dehydrogenaseAdd BLAST450

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

eggNOGiKOG0172. Eukaryota.
COG1748. LUCA.
GeneTreeiENSGT00390000013249.
HOGENOMiHOG000252920.
HOVERGENiHBG048688.
InParanoidiQ99K67.
KOiK14157.
OMAiYGPEEKD.
OrthoDBiEOG091G03D7.
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.
IPR032095. Sacchrp_dh_C.
IPR005097. Sacchrp_dh_NADP.
[Graphical view]
PfamiPF05222. AlaDh_PNT_N. 1 hit.
PF16653. Sacchrp_dh_C. 1 hit.
PF03435. Sacchrp_dh_NADP. 1 hit.
[Graphical view]
SMARTiSM01002. AlaDh_PNT_C. 1 hit.
SM01003. AlaDh_PNT_N. 1 hit.
[Graphical view]
SUPFAMiSSF51735. SSF51735. 1 hit.

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)DescriptionActionsGraphical viewLength
Sequence conflicti198V → I in CAA12114 (PubMed:10567240).Curated1
Sequence conflicti851L → A in CAA12114 (PubMed:10567240).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
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:
EMBLi
GenBanki
DDBJi
Links Updated
AJ224761 mRNA. Translation: CAA12114.1.
BC005420 mRNA. Translation: AAH05420.1.
CCDSiCCDS19937.1.
RefSeqiNP_038958.2. NM_013930.4.
UniGeneiMm.18651.

3D structure databases

ProteinModelPortaliQ99K67.
SMRiQ99K67.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiQ99K67. 2 interactors.
MINTiMINT-1840773.
STRINGi10090.ENSMUSP00000031707.

PTM databases

iPTMnetiQ99K67.
PhosphoSitePlusiQ99K67.

2D gel databases

REPRODUCTION-2DPAGEQ99K67.

Proteomic databases

MaxQBiQ99K67.
PaxDbiQ99K67.
PRIDEiQ99K67.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi10157.
MGIiMGI:1353573. Aass.

Phylogenomic databases

eggNOGiKOG0172. Eukaryota.
COG1748. LUCA.
GeneTreeiENSGT00390000013249.
HOGENOMiHOG000252920.
HOVERGENiHBG048688.
InParanoidiQ99K67.
KOiK14157.
OMAiYGPEEKD.
OrthoDBiEOG091G03D7.
PhylomeDBiQ99K67.
TreeFamiTF105728.

Enzyme and pathway databases

UniPathwayiUPA00868; UER00835.
UPA00868; UER00836.
ReactomeiR-MMU-71064. Lysine catabolism.

Miscellaneous databases

PROiQ99K67.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000029695.
CleanExiMM_AASS.
ExpressionAtlasiQ99K67. baseline and differential.
GenevisibleiQ99K67. MM.

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.
IPR032095. Sacchrp_dh_C.
IPR005097. Sacchrp_dh_NADP.
[Graphical view]
PfamiPF05222. AlaDh_PNT_N. 1 hit.
PF16653. Sacchrp_dh_C. 1 hit.
PF03435. Sacchrp_dh_NADP. 1 hit.
[Graphical view]
SMARTiSM01002. AlaDh_PNT_C. 1 hit.
SM01003. AlaDh_PNT_N. 1 hit.
[Graphical view]
SUPFAMiSSF51735. SSF51735. 1 hit.
ProtoNetiSearch...

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: November 30, 2016
This is version 124 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

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 one 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.