NUCLEOTIDE SEQUENCE [MRNA].
STRAIN=C57BL/6J X CBA/J;
TISSUE=Liver;
DOI=10.1042/0264-6021:3440555; PubMed=10567240 [NCBI, ExPASy, EBI, Israel, Japan]
Papes F., Kemper E.L., Cord-Neto G., Langone F., Arruda P.;
"Lysine degradation through the saccharopine pathway in mammals: involvement of both bifunctional and monofunctional lysine-degrading enzymes in mouse.";
Biochem. J. 344:555-563(1999).

[2]

NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
TISSUE=Mammary tumor;
DOI=10.1101/gr.2596504; PubMed=15489334 [NCBI, ExPASy, EBI, Israel, Japan]
The MGC Project Team;
"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).";
Genome Res. 14:2121-2127(2004).

Comments

FUNCTION: 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 ACTIVITY: N⁶-(L-1,3-dicarboxypropyl)-L-lysine + NADP⁺ + H₂O = L-lysine + 2-oxoglutarate + NADPH.
CATALYTIC ACTIVITY: N⁶-(L-1,3-dicarboxypropyl)-L-lysine + NAD⁺ + H₂O = L-glutamate + 2-aminoadipate 6-semialdehyde + NADH.
PATHWAY: Amino-acid degradation; L-lysine degradation via saccharopine pathway; glutaryl-CoA from L-lysine: step 1/6 .
PATHWAY: Amino-acid degradation; L-lysine degradation via saccharopine pathway; glutaryl-CoA from L-lysine: step 2/6 .
SUBUNIT: Homodimer.
SUBCELLULAR LOCATION: Mitochondrion.
TISSUE SPECIFICITY: 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.
INDUCTION: Induced by starvation.
SIMILARITY: In the N-terminal section; belongs to the AlaDH/PNT family.
SIMILARITY: In the C-terminal section; belongs to the saccharopine dehydrogenase family.

Copyright

Copyrighted by the UniProt Consortium, see http://www.uniprot.org/terms. Distributed under the Creative Commons Attribution-NoDerivs License.

Cross-references

Sequence databases

EMBL

AJ224761; CAA12114.1; -; mRNA.	[EMBL / GenBank / DDBJ] [CoDingSequence]
BC005420; AAH05420.1; -; mRNA.	[EMBL / GenBank / DDBJ] [CoDingSequence]

RefSeq

NP_038958.2; -.

UniGene

Mm.18651

3D structure databases

ModBase

Q99K67.

2D gel databases

REPRODUCTION-2DPAGE

Q99K67; -.

Organism-specific databases

MGI

MGI:1353573; Aass.

Gene expression databases

ArrayExpress

Q99K67; -.

CleanEx

MM_AASS; -.

GermOnline

ENSMUSG00000029695; Mus musculus.

Ontologies

GO:0005739;	Cellular component: mitochondrion (traceable author statement from UniProtKB).
GO:0047131;	Molecular function: saccharopine dehydrogenase (NAD+, L-glutamate-forming) activity (inferred from direct assay from UniProtKB).
GO:0006091;	Biological process: generation of precursor metabolites and energy (traceable author statement from UniProtKB).
QuickGo view.

Family and domain databases

InterPro

IPR007698; Ala_DHase/PNT_C.
IPR008142; Ala_DHase/PNT_CS1.
IPR008143; Ala_DHase/PNT_CS2.
IPR007886; Ala_DHase/PNT_N.
IPR005097; Saccharopine_DHase.
Graphical view of domain structure.

Pfam

PF01262; AlaDh_PNT_C; 1.
PF05222; AlaDh_PNT_N; 1.
PF03435; Saccharop_dh; 1.
Pfam graphical view of domain structure.

PROSITE

PS00836; ALADH_PNT_1; FALSE_NEG.
PS00837; ALADH_PNT_2; FALSE_NEG.

BLOCKS

Q99K67.

Genome annotation databases

Ensembl

ENSMUSG00000029695; Mus musculus. [Contig view]

GeneID

30956; -.

KEGG

mmu:30956; -.

Phylogenomic databases

HOGENOM

Q99K67; -.

HOVERGEN

Q99K67; -.

Other

Aass; Mus musculus.

View cluster of proteins with at least 50% / 90% / 100% identity.

Keywords

Mitochondrion; Multifunctional enzyme; NAD; NADP; Oxidoreductase; Transit peptide.

Features

Feature table viewer

Feature aligner

`Key`	`From To`	`Length`	`Description`	`FTId`
`TRANSIT`	`1 32`	`32`	`Mitochondrion.`
`CHAIN`	`33 926`	`894`	`Alpha-aminoadipic semialdehyde synthase, mitochondrial.`	`PRO_0000001053`
`REGION`	`33 455`	`423`	`Lysine-ketoglutarate reductase.`
`REGION`	`477 926`	`450`	`Saccharopine dehydrogenase.`
`CONFLICT`	`198 198`		`V -> I (in Ref. 1; CAA12114).`
`CONFLICT`	`851 851`		`L -> A (in Ref. 1; CAA12114).`

Sequence information

Length: 926 AA [This is the length of the unprocessed precursor]

Molecular weight: 102975 Da [This is the MW of the unprocessed precursor]

CRC64: 5B4369C51F7D1D53 [This is a checksum on the sequence]

        10         20         30         40         50         60 
MLRAQRPRLA RLRACLSRGL HHKPVMALRR EDVNAWERRA PLAPKHIKGI TKLGYKVLIQ 

        70         80         90        100        110        120 
PSNRRAIHDK EYVRAGGILQ EDITEACLIL GVKRPPEEKL MSKKTYAFFS HTIKAQEANM 

       130        140        150        160        170        180 
NLLDEVLKQE IRLIDYEKMV DHRGSRIVAF GQWAGVAGMI NILHGMGLRL LALGHHTPFM 

       190        200        210        220        230        240 
HLGMAHNYRN SSQAVQAVRD AGYEISLGLM PKSIGPLTFV FTGTGNVSKG AQEVFNELPC 

       250        260        270        280        290        300 
EYVEPHELRE VSKTGDLRKV YGTVLSRHHH LVRKTDGVYD PVEYEKYPER YTSRFNTDIA 

       310        320        330        340        350        360 
PYTTCLINGI YWEQNTPRLL TRQDAQSLLV PVKSSVVPVE GCPELPHKLV AICDISADTG 

       370        380        390        400        410        420 
GSIDFMTECT TIERPFCMYD ADQQIIHDSV EGSGILMCSI DNLPAQLPIE ATEYFGDMLY 

       430        440        450        460        470        480 
PYVEEMLLSD ASQPLESQNF SPVVRDAVIT SNGLLTDKYK YIQKLRESRE RIQFLSMSTK 

       490        500        510        520        530        540 
KKVLVLGSGY VSGPVLEYLS RDNNIEITLG SDMTNQMQQL SKKYNINPVS LTVGKQEAKL 

       550        560        570        580        590        600 
QSLVESQDLV ISLLPYVLHP VVAKACIESR VNMVTASYIT PAMKELEKSV DDAGITVIGE 

       610        620        630        640        650        660 
LGLDPGLDHM LAMETIDTAK ELGATVESYV SYCGGLPAPE HSDNPLRYKF SWSPVGVLMN 

       670        680        690        700        710        720 
IMQPASYLLN GKVVNVTGGV SFLNSVTPMD YFPGLNLEGY PNRDSIKYAE IYGISSAHTL 

       730        740        750        760        770        780 
LRGTLRYKGY SKALNGFVKL GLINREAYPA LRPEANPLTW KQLLCDLVGI SRSSPCEKLK 

       790        800        810        820        830        840 
EVVFTKLGGD NTQLEAAEWL GLLGDEQVPQ AESIVDAFSK HLVSKLSYGP EEKDMIVMRD 

       850        860        870        880        890        900 
SFGIRHPSGH LENKTIDLVV YGDFNGFSAM AKTVGLPTAM AAKMLLDGEI EAKGLMGPFT 

       910        920 
KEIYGPILER IKAEGIVFNT QSTIKL

Q99K67 in FASTA format

View entry in original UniProtKB/Swiss-Prot format
View entry in raw text format (no links)
Report form for errors/updates in this UniProtKB/Swiss-Prot entry

	BLAST submission on ExPASy/SIB or at NCBI (USA)		Sequence analysis tools: ProtParam, ProtScale, Compute pI/Mw, PeptideMass, PeptideCutter, Dotlet (Java)
	ScanProsite, MotifScan		Submit a homology modeling request to SWISS-MODEL
	NPSA Sequence analysis tools