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Protein

Beta-alanine-activating enzyme

Gene

Aasdh

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

Functioni

Covalently binds beta-alanine in an ATP-dependent manner to form a thioester bond with its phosphopantetheine group and transfers it to an as yet unknown acceptor via an amide bond. May be required for a post-translational protein modification or for post-transcriptional modification of an RNA.1 Publication1 Publication

Caution

Was suggested to bind pyrroloquinoline quinone (PQQ) based on prediction tools and indirect results (PubMed:12712191). However, this has not been confirmed in other publications (PubMed:15689995, PubMed:15689994).3 Publications
In invertebrates, the aminoadipate-semialdehyde dehydrogenase reaction is a key step of the L-lysine biosynthesis pathway which is not fully conserved in vertebrates and it has been suggested that this protein participates in the reverse reaction i.e. in lysine catabolism (PubMed:12712191). However, this is unlikely to be the case as no dehydrogenase activity has been detected and the authentic mammalian 2-aminoadipate semialdehyde dehydrogenase has been identified as ALDH7A1 (PubMed:24467666).2 Publications

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei427ATPBy similarity1
Binding sitei441ATPBy similarity1
Binding sitei526ATPBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi197 – 205ATPBy similarity9

GO - Molecular functioni

GO - Biological processi

  • amino acid activation for nonribosomal peptide biosynthetic process Source: MGI
  • beta-alanine metabolic process Source: MGI
  • fatty acid metabolic process Source: UniProtKB

Keywordsi

Molecular functionLigase
Biological processFatty acid metabolism, Lipid metabolism
LigandATP-binding, NAD, Nucleotide-binding

Names & Taxonomyi

Protein namesi
Recommended name:
Beta-alanine-activating enzyme1 Publication (EC:6.2.1.-)
Alternative name(s):
Acyl-CoA synthetase family member 4
Protein LYS2 homolog
Gene namesi
Name:Aasdh
Synonyms:Acsf4, U26
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 5

Organism-specific databases

MGIiMGI:2442517 Aasdh

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi591S → A: Abolishes incorporation of beta-alanine. 1 Publication1

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00003158041 – 1100Beta-alanine-activating enzymeAdd BLAST1100

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei591O-(pantetheine 4'-phosphoryl)serinePROSITE-ProRule annotation1
Modified residuei651PhosphoserineBy similarity1

Keywords - PTMi

Phosphopantetheine, Phosphoprotein

Proteomic databases

PaxDbiQ80WC9
PRIDEiQ80WC9

PTM databases

iPTMnetiQ80WC9
PhosphoSitePlusiQ80WC9

Expressioni

Inductioni

Has been shown to be up-regulated by a lysine-rich diet (PubMed:12712191). However, levels of expression have also been shown not to be significantly changed even when diets differ markedly in PQQ and lysine content (PubMed:17029795).2 Publications

Gene expression databases

BgeeiENSMUSG00000055923
CleanExiMM_AASDH
ExpressionAtlasiQ80WC9 baseline and differential
GenevisibleiQ80WC9 MM

Interactioni

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000069279

Structurei

3D structure databases

ProteinModelPortaliQ80WC9
SMRiQ80WC9
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini552 – 632CarrierPROSITE-ProRule annotationAdd BLAST81

Sequence similaritiesi

Phylogenomic databases

eggNOGiKOG1178 Eukaryota
KOG4649 Eukaryota
COG1520 LUCA
GeneTreeiENSGT00440000033811
HOGENOMiHOG000033793
HOVERGENiHBG057704
InParanoidiQ80WC9
KOiK00142
OMAiCYNGLVY
OrthoDBiEOG091G05QW
PhylomeDBiQ80WC9
TreeFamiTF314245

Family and domain databases

Gene3Di2.130.10.10, 2 hits
InterProiView protein in InterPro
IPR036736 ACP-like_sf
IPR020845 AMP-binding_CS
IPR000873 AMP-dep_Synth/Lig
IPR009081 PP-bd_ACP
IPR006162 Ppantetheine_attach_site
IPR025666 PQQ-like_dom
IPR018391 PQQ_beta_propeller_repeat
IPR002372 PQQ_repeat
IPR011047 Quinoprotein_ADH-like_supfam
IPR015943 WD40/YVTN_repeat-like_dom_sf
PfamiView protein in Pfam
PF00501 AMP-binding, 1 hit
PF00550 PP-binding, 1 hit
PF13360 PQQ_2, 1 hit
PF13570 PQQ_3, 1 hit
SMARTiView protein in SMART
SM00564 PQQ, 6 hits
SUPFAMiSSF47336 SSF47336, 1 hit
SSF50998 SSF50998, 1 hit
PROSITEiView protein in PROSITE
PS00455 AMP_BINDING, 1 hit
PS50075 CARRIER, 1 hit
PS00012 PHOSPHOPANTETHEINE, 1 hit

Sequences (3)i

Sequence statusi: Complete.

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q80WC9-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MTLQELVLRT ASVYMDRTAV CFDEGNNQPP VCYSYKALLS AASELSHFLI
60 70 80 90 100
AHCDFGGIRE IGLYCQPGIN LPSWILGILQ VPAAYAPIDP DSPPSLSTYF
110 120 130 140 150
MKKCDLKYVL VEKQQLSKFK SSHETVLNYD TVSVEHKDLA LFRLHWEDGR
160 170 180 190 200
VSTVLGDRAD QHKVTDREDR VSAESRTPEK EHMDMRHDGC LAYVLHTSGT
210 220 230 240 250
TGTPKIVRVP HACILPNIQH FRSLFDITQE DILFLASPLT FDPSVVEIFV
260 270 280 290 300
SLSSGACLLI VPTSVKVLPS KLADILFSRH RVTVLQATPT LLRRFGSELI
310 320 330 340 350
KSTVLSAHTS LRVLALGGEA FPSLTILKSW RGKGNRTQIF NIYGITEVSS
360 370 380 390 400
WATFYRIPEE ILNSAVKHES PVQLGSPLLG TVIEVRDQNG SPVLEGTGQV
410 420 430 440 450
FLGGKNRVCF LDDEMTVPLG TMRATGDFVT VKDGEIFFLG RKDSQIKRHG
460 470 480 490 500
KRLNIALVQQ VAEELRQVES CAVTWYNQER LILFIVSKVD LVKDCIFKEL
510 520 530 540 550
QKHLPAHALP DDMVLIDTLP FTCHGKVDVS ELNKIYLDYI SSQPRNELHG
560 570 580 590 600
KEELWGKLQY LWKSILCLPE DPEDTLKVPA NSVFLDSGGD SLKSMRLLSE
610 620 630 640 650
IERLTGTAIP GLLEVILSSS LLDVYNHIVQ AVFTPEDRKA NRSYTTKRKF
660 670 680 690 700
SDADPEEASG KPARLESAWP SNHAGETNSV IALSRGSQVL SLGAGRLLTQ
710 720 730 740 750
LGLCLPVCSL DLIPQTNTQI LKSLSPPAPD ENLEKPPLFQ QGSPVVGAMA
760 770 780 790 800
MALRERWRSD TGKCVDASPL LVRAAVQDKP STTVYIGSHS HTVKAVDLSS
810 820 830 840 850
GETRWEQLLG DRIESSACVS KCGNFIVVGC YNGLVYVLKS NSGEKYWTFT
860 870 880 890 900
TEDAVKSSPA VDPTTGLIYV GSHDQHAYAL DIYEKKCVWK LNCEGALFSS
910 920 930 940 950
PCVSLSPHHL YCATLGGLLL ALNPASGSTV WKRSCGKPLF SSPRCYQQYI
960 970 980 990 1000
CIGCVDGSLL CFTHSGEQVW RFAAGGPIFS SPCVSAAEQE IFFGSHDCFI
1010 1020 1030 1040 1050
YCCSKEGHLR WKFETTARVY ATPFAFSNHP RSDDALLAAA STDGKLWVLE
1060 1070 1080 1090 1100
SRSGELRSVY ELPGEVFSSP VVWESMLVIG CRNNYIYCLD LLCGDKNNQV
Length:1,100
Mass (Da):121,569
Last modified:June 1, 2003 - v1
Checksum:i86B303CFF07B234C
GO
Isoform 2 (identifier: Q80WC9-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     368-1100: Missing.

Note: No experimental confirmation available.
Show »
Length:367
Mass (Da):40,964
Checksum:i19368BB77FDDF580
GO
Isoform 3 (identifier: Q80WC9-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     223-1100: Missing.

Note: No experimental confirmation available.
Show »
Length:222
Mass (Da):25,031
Checksum:i2850F469893CD77F
GO

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_030714223 – 1100Missing in isoform 3. 1 PublicationAdd BLAST878
Alternative sequenceiVSP_030715368 – 1100Missing in isoform 2. 1 PublicationAdd BLAST733

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB095954 mRNA Translation: BAC75954.1
AK038779 mRNA Translation: BAE43303.1
AK043807 mRNA Translation: BAC31660.1
BC094507 mRNA Translation: AAH94507.1
BC128330 mRNA Translation: AAI28331.1
CCDSiCCDS19366.1 [Q80WC9-1]
RefSeqiNP_776126.1, NM_173765.3 [Q80WC9-1]
XP_006534916.1, XM_006534853.2 [Q80WC9-1]
XP_011247723.1, XM_011249421.1 [Q80WC9-1]
XP_011247724.1, XM_011249422.1 [Q80WC9-1]
UniGeneiMm.39271

Genome annotation databases

EnsembliENSMUST00000069709; ENSMUSP00000069279; ENSMUSG00000055923 [Q80WC9-1]
ENSMUST00000120963; ENSMUSP00000113792; ENSMUSG00000055923 [Q80WC9-1]
GeneIDi231326
KEGGimmu:231326
UCSCiuc008xvi.2 mouse [Q80WC9-1]
uc008xvk.2 mouse [Q80WC9-3]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiACSF4_MOUSE
AccessioniPrimary (citable) accession number: Q80WC9
Secondary accession number(s): Q3V3L2, Q505K4, Q8BRP4
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 15, 2008
Last sequence update: June 1, 2003
Last modified: April 25, 2018
This is version 122 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
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Main funding by: National Institutes of Health