Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Alpha-aminoadipic semialdehyde synthase, mitochondrial

Gene

Aass

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

N6-(L-1,3-dicarboxypropyl)-L-lysine + NADP+ + H2O = L-lysine + 2-oxoglutarate + NADPH.
N6-(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.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei604SaccharopineBy similarity1
Binding sitei653NADP; via amide nitrogenBy similarity1
Binding sitei703SaccharopineBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi488 – 491NADPBy similarity4
Nucleotide bindingi603 – 605NADPBy similarity3

GO - Molecular functioni

  • saccharopine dehydrogenase (NAD+, L-glutamate-forming) activity Source: UniProtKB
  • saccharopine dehydrogenase (NADP+, L-lysine-forming) activity Source: MGI
  • saccharopine dehydrogenase activity Source: GO_Central

GO - Biological processi

Keywordsi

Molecular functionMultifunctional enzyme, Oxidoreductase
LigandNAD, NADP

Enzyme and pathway databases

ReactomeiR-MMU-71064 Lysine catabolism
UniPathwayi
UPA00868;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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 6

Organism-specific databases

MGIiMGI:1353573 Aass

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

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-2DPAGEiQ99K67

PTM databases

iPTMnetiQ99K67
PhosphoSitePlusiQ99K67
SwissPalmiQ99K67

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 Expressed in 220 organ(s), highest expression level in proximal tubule
CleanExiMM_AASS
ExpressionAtlasiQ99K67 baseline and differential
GenevisibleiQ99K67 MM

Interactioni

Subunit structurei

Homodimer.

Protein-protein interaction databases

IntActiQ99K67, 2 interactors
MINTiQ99K67
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
Regioni577 – 578Saccharopine bindingBy similarity2
Regioni724 – 726Saccharopine bindingBy similarity3

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
OMAiKVNMITA
OrthoDBiEOG091G03D7
PhylomeDBiQ99K67
TreeFamiTF105728

Family and domain databases

InterProiView protein in InterPro
IPR007886 AlaDH/PNT_N
IPR007698 AlaDH/PNT_NAD(H)-bd
IPR036291 NAD(P)-bd_dom_sf
IPR032095 Sacchrp_dh_C
IPR005097 Sacchrp_dh_NADP
PfamiView protein in Pfam
PF05222 AlaDh_PNT_N, 1 hit
PF16653 Sacchrp_dh_C, 1 hit
PF03435 Sacchrp_dh_NADP, 1 hit
SMARTiView protein in SMART
SM01002 AlaDh_PNT_C, 1 hit
SM01003 AlaDh_PNT_N, 1 hit
SUPFAMiSSF51735 SSF51735, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

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

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All

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

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
F6VMP2F6VMP2_MOUSE
Alpha-aminoadipic semialdehyde synt...
Aass
209Annotation score:

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

Similar proteinsi

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
MINTiQ99K67
STRINGi10090.ENSMUSP00000031707

PTM databases

iPTMnetiQ99K67
PhosphoSitePlusiQ99K67
SwissPalmiQ99K67

2D gel databases

REPRODUCTION-2DPAGEiQ99K67

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
OMAiKVNMITA
OrthoDBiEOG091G03D7
PhylomeDBiQ99K67
TreeFamiTF105728

Enzyme and pathway databases

UniPathwayi
UPA00868;UER00835

UPA00868;UER00836

ReactomeiR-MMU-71064 Lysine catabolism

Miscellaneous databases

PROiPR:Q99K67
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000029695 Expressed in 220 organ(s), highest expression level in proximal tubule
CleanExiMM_AASS
ExpressionAtlasiQ99K67 baseline and differential
GenevisibleiQ99K67 MM

Family and domain databases

InterProiView protein in InterPro
IPR007886 AlaDH/PNT_N
IPR007698 AlaDH/PNT_NAD(H)-bd
IPR036291 NAD(P)-bd_dom_sf
IPR032095 Sacchrp_dh_C
IPR005097 Sacchrp_dh_NADP
PfamiView protein in Pfam
PF05222 AlaDh_PNT_N, 1 hit
PF16653 Sacchrp_dh_C, 1 hit
PF03435 Sacchrp_dh_NADP, 1 hit
SMARTiView protein in SMART
SM01002 AlaDh_PNT_C, 1 hit
SM01003 AlaDh_PNT_N, 1 hit
SUPFAMiSSF51735 SSF51735, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiAASS_MOUSE
AccessioniPrimary (citable) accession number: Q99K67
Secondary accession number(s): Q9Z1I9
Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 13, 2004
Last sequence update: June 1, 2001
Last modified: November 7, 2018
This is version 136 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  3. PATHWAY comments
    Index of metabolic and biosynthesis pathways
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.

Do not show this banner again