U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Aasdh aminoadipate-semialdehyde dehydrogenase [ Mus musculus (house mouse) ]

Gene ID: 231326, updated on 27-Nov-2024

Summary

Go to the top of the page Help
Official Symbol
Aasdhprovided by MGI
Official Full Name
aminoadipate-semialdehyde dehydrogenaseprovided by MGI
Primary source
MGI:MGI:2442517
See related
Ensembl:ENSMUSG00000055923 AllianceGenome:MGI:2442517
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Mus musculus
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as
U26; Acsf4; A830035E16; A230062G08Rik
Summary
The gene product is a cytosolic enzyme involved in the production of alpha-aminoadipic acid from alpha-aminoadipic semialdehyde. It is postulated that this enzyme plays a role in lysine metabolism. There is currently debate regarding this enzyme's putative requirement of pyrroloquinoline quinine as an essential cofactor. A related pseudogene has been identified on chromosome 2. [provided by RefSeq, Jan 2010]
Expression
Ubiquitous expression in bladder adult (RPKM 4.3), CNS E14 (RPKM 3.1) and 28 other tissues See more
Orthologs
human all
NEW
Try the new Gene table
Try the new Transcript table

Genomic context

Go to the top of the page Help
See Aasdh in Genome Data Viewer
Location:
5 C3.3; 5 41.45 cM
Exon count:
21
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCm39 (GCF_000001635.27) 5 NC_000071.7 (77021506..77053376, complement)
108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 5 NC_000071.6 (76873659..76905529, complement)

Chromosome 5 - NC_000071.7Genomic Context describing neighboring genes Neighboring gene capping protein inhibiting regulator of actin Neighboring gene predicted gene, 34369 Neighboring gene STARR-seq mESC enhancer starr_13449 Neighboring gene STARR-seq mESC enhancer starr_13451 Neighboring gene predicted gene, 52818 Neighboring gene STARR-seq mESC enhancer starr_13452 Neighboring gene phosphoribosyl pyrophosphate amidotransferase Neighboring gene phosphoribosylaminoimidazole carboxylase, phosphoribosylaminoribosylaminoimidazole, succinocarboxamide synthetase Neighboring gene predicted gene, 54291

Genomic regions, transcripts, and products

Go to the top of the page Help

Go to nucleotide: Graphics FASTA GenBank

Expression

Go to the top of the page Help
  • Project title: Mouse ENCODE transcriptome data
  • Description: RNA profiling data sets generated by the Mouse ENCODE project.
  • BioProject: PRJNA66167
  • Publication: PMID 25409824
  • Analysis date: n/a

Bibliography

Go to the top of the pageHelp

Related articles in PubMed

  1. Biochemistry: is pyrroloquinoline quinone a vitamin? Rucker R, et al. Nature, 2005 Feb 3. PMID 15689994
  2. Biochemistry: role of PQQ as a mammalian enzyme cofactor? Felton LM, et al. Nature, 2005 Feb 3. PMID 15689995
  3. Pyrroloquinoline quinone nutritional status alters lysine metabolism and modulates mitochondrial DNA content in the mouse and rat. Bauerly KA, et al. Biochim Biophys Acta, 2006 Nov. PMID 17029795
  4. Cloning and characterization of a novel human homolog* of mouse U26, a putative PQQ-dependent AAS dehydrogenase. Wang L, et al. Mol Biol Rep, 2005 Mar. PMID 15865210
  5. Nutritional biochemistry: A new redox-cofactor vitamin for mammals. Kasahara T, et al. Nature, 2003 Apr 24. PMID 12712191

See all (21) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. Recombinant ACSF4-U26 progressively formed a covalent bond with radiolabelled beta-alanine. The bond was not formed in a point mutant lacking the phosphopantetheine attachment site.
    Title: Vertebrate Acyl CoA synthetase family member 4 (ACSF4-U26) is a β-alanine-activating enzyme homologous to bacterial non-ribosomal peptide synthetase.
  2. ACSF4 (U26) is a beta-alanine activating enzyme homologous to bacterial nonribosomal peptide synthetase. Experiments using the mouse protein suggest this enzyme may be involved in a rare intracellular reaction, possibly an infrequent post-translational or post-transcriptional modification.
    Title: Vertebrate Acyl CoA synthetase family member 4 (ACSF4-U26) is a β-alanine-activating enzyme homologous to bacterial non-ribosomal peptide synthetase.
  3. involved in the lysine degradation pathway
    Title: Nutritional biochemistry: A new redox-cofactor vitamin for mammals.
Submit: New GeneRIF Correction

Variation

Go to the top of the page Help

Alleles

Alleles of this type are documented at Mouse Genome Informatics  (MGI)
  • Endonuclease-mediated (2) 
  • Gene trapped (1) 

Interactions

Go to the top of the page Help
Products Interactant Other Gene Complex Source Pubs Description

General gene information

Go to the top of the page Help

Markers

Homology

Gene Ontology Provided by MGI

Function Evidence Code Pubs
enables ATP binding IEA
Inferred from Electronic Annotation
more info
  
enables acid-thiol ligase activity ISS
Inferred from Sequence or Structural Similarity
more info
  
enables molecular_function ND
No biological Data available
more info
  
Process Evidence Code Pubs
involved_in amino acid activation for nonribosomal peptide biosynthetic process IBA
Inferred from Biological aspect of Ancestor
more info
  
acts_upstream_of_or_within amino acid activation for nonribosomal peptide biosynthetic process IDA
Inferred from Direct Assay
more info
 PubMed 
acts_upstream_of_or_within beta-alanine metabolic process IDA
Inferred from Direct Assay
more info
 PubMed 
involved_in fatty acid metabolic process ISS
Inferred from Sequence or Structural Similarity
more info
  
Component Evidence Code Pubs
is_active_in cellular_component ND
No biological Data available
more info
  

General protein information

Go to the top of the page Help
Preferred Names
beta-alanine-activating enzyme
Names
2-aminoadipic 6-semialdehyde dehydrogenase
acyl-CoA synthetase family member 4
protein LYS2 homolog
putative aminoadipate-semialdehyde dehydrogenase
NP_001363933.1
NP_776126.1
XP_006534916.1
XP_006534917.1
XP_006534919.1
XP_006534920.1
XP_006534922.1
XP_006534923.1
XP_011247724.1
XP_011247725.1
XP_017176330.1
XP_030110250.1
XP_030110251.1
XP_030110252.1
XP_036020953.1
XP_036020954.1
XP_036020955.1
XP_036020956.1
XP_036020957.1

NCBI Reference Sequences (RefSeq)

Go to the top of the page Help

NEW Try the new Transcript table

RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

  1. NM_001377004.1 → NP_001363933.1  beta-alanine-activating enzyme

    Status: REVIEWED

    Description
    Transcript Variant: Variants 1 and 2 encode the same protein.
    Source sequence(s)
    AC114666, AC166328
    UniProtKB/Swiss-Prot
    Q3V3L2, Q505K4, Q80WC9, Q8BRP4
    Conserved Domains (4) summary
    COG1520
    Location:754 → 986
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    TIGR03300
    Location:923 → 1094
    assembly_YfgL; outer membrane assembly lipoprotein YfgL
    cd17654
    Location:15 → 532
    A_NRPS_acs4; acyl-CoA synthetase family member 4
    pfam00550
    Location:561 → 628
    PP-binding; Phosphopantetheine attachment site

  2. NM_173765.4 → NP_776126.1  beta-alanine-activating enzyme

    See identical proteins and their annotated locations for NP_776126.1

    Status: REVIEWED

    Description
    Transcript Variant: Variants 1 and 2 encode the same protein.
    Source sequence(s)
    AC114666, AC166328
    Consensus CDS
    CCDS19366.1
    UniProtKB/Swiss-Prot
    Q3V3L2, Q505K4, Q80WC9, Q8BRP4
    Related
    ENSMUSP00000113792.2, ENSMUST00000120963.8
    Conserved Domains (4) summary
    COG1520
    Location:754 → 986
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    TIGR03300
    Location:923 → 1094
    assembly_YfgL; outer membrane assembly lipoprotein YfgL
    cd17654
    Location:15 → 532
    A_NRPS_acs4; acyl-CoA synthetase family member 4
    pfam00550
    Location:561 → 628
    PP-binding; Phosphopantetheine attachment site

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

  1. NC_000071.7 Reference GRCm39 C57BL/6J

    Range
    77021506..77053376 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_006534854.3 → XP_006534917.1  beta-alanine-activating enzyme isoform X2

    Conserved Domains (4) summary
    COG1520
    Location:754 → 1051
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    sd00039
    Location:815 → 849
    7WD40; WD40 repeat [structural motif]
    cd17654
    Location:15 → 532
    A_NRPS_acs4; acyl-CoA synthetase family member 4
    pfam00550
    Location:561 → 628
    PP-binding; Phosphopantetheine attachment site

  2. XM_011249423.3 → XP_011247725.1  beta-alanine-activating enzyme isoform X4

    Conserved Domains (4) summary
    TIGR03300
    Location:730 → 881
    assembly_YfgL; outer membrane assembly lipoprotein YfgL
    sd00039
    Location:816 → 850
    7WD40; WD40 repeat [structural motif]
    cd17654
    Location:15 → 532
    A_NRPS_acs4; acyl-CoA synthetase family member 4
    pfam00550
    Location:561 → 628
    PP-binding; Phosphopantetheine attachment site

  3. XM_006534856.4 → XP_006534919.1  beta-alanine-activating enzyme isoform X5

    Conserved Domains (4) summary
    TIGR03300
    Location:730 → 881
    assembly_YfgL; outer membrane assembly lipoprotein YfgL
    sd00039
    Location:816 → 850
    7WD40; WD40 repeat [structural motif]
    cd17654
    Location:15 → 532
    A_NRPS_acs4; acyl-CoA synthetase family member 4
    pfam00550
    Location:561 → 628
    PP-binding; Phosphopantetheine attachment site

  4. XM_006534857.4 → XP_006534920.1  beta-alanine-activating enzyme isoform X6

    Conserved Domains (4) summary
    COG1520
    Location:592 → 824
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    TIGR03300
    Location:761 → 932
    assembly_YfgL; outer membrane assembly lipoprotein YfgL
    cd17654
    Location:26 → 370
    A_NRPS_acs4; acyl-CoA synthetase family member 4
    pfam00550
    Location:399 → 448
    PP-binding; Phosphopantetheine attachment site

  5. XM_036165061.1 → XP_036020954.1  beta-alanine-activating enzyme isoform X8

    Conserved Domains (4) summary
    COG1520
    Location:569 → 866
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    sd00039
    Location:630 → 664
    7WD40; WD40 repeat [structural motif]
    cd17654
    Location:34 → 347
    A_NRPS_acs4; acyl-CoA synthetase family member 4
    pfam00550
    Location:376 → 425
    PP-binding; Phosphopantetheine attachment site

  6. XM_030254392.1 → XP_030110252.1  beta-alanine-activating enzyme isoform X7

    Conserved Domains (4) summary
    COG1520
    Location:569 → 801
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    TIGR03300
    Location:738 → 909
    assembly_YfgL; outer membrane assembly lipoprotein YfgL
    cd17654
    Location:34 → 347
    A_NRPS_acs4; acyl-CoA synthetase family member 4
    pfam00550
    Location:376 → 443
    PP-binding; Phosphopantetheine attachment site

  7. XM_006534853.3 → XP_006534916.1  beta-alanine-activating enzyme isoform X1

    See identical proteins and their annotated locations for XP_006534916.1

    UniProtKB/Swiss-Prot
    Q3V3L2, Q505K4, Q80WC9, Q8BRP4
    Related
    ENSMUSP00000069279.9, ENSMUST00000069709.15
    Conserved Domains (4) summary
    COG1520
    Location:754 → 986
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    TIGR03300
    Location:923 → 1094
    assembly_YfgL; outer membrane assembly lipoprotein YfgL
    cd17654
    Location:15 → 532
    A_NRPS_acs4; acyl-CoA synthetase family member 4
    pfam00550
    Location:561 → 628
    PP-binding; Phosphopantetheine attachment site

  8. XM_011249422.2 → XP_011247724.1  beta-alanine-activating enzyme isoform X1

    See identical proteins and their annotated locations for XP_011247724.1

    UniProtKB/Swiss-Prot
    Q3V3L2, Q505K4, Q80WC9, Q8BRP4
    Conserved Domains (4) summary
    COG1520
    Location:754 → 986
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    TIGR03300
    Location:923 → 1094
    assembly_YfgL; outer membrane assembly lipoprotein YfgL
    cd17654
    Location:15 → 532
    A_NRPS_acs4; acyl-CoA synthetase family member 4
    pfam00550
    Location:561 → 628
    PP-binding; Phosphopantetheine attachment site

  9. XM_036165060.1 → XP_036020953.1  beta-alanine-activating enzyme isoform X6

    Conserved Domains (4) summary
    COG1520
    Location:592 → 824
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    TIGR03300
    Location:761 → 932
    assembly_YfgL; outer membrane assembly lipoprotein YfgL
    cd17654
    Location:26 → 370
    A_NRPS_acs4; acyl-CoA synthetase family member 4
    pfam00550
    Location:399 → 448
    PP-binding; Phosphopantetheine attachment site

  10. XM_030254390.1 → XP_030110250.1  beta-alanine-activating enzyme isoform X2

    Conserved Domains (4) summary
    COG1520
    Location:754 → 1051
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    sd00039
    Location:815 → 849
    7WD40; WD40 repeat [structural motif]
    cd17654
    Location:15 → 532
    A_NRPS_acs4; acyl-CoA synthetase family member 4
    pfam00550
    Location:561 → 628
    PP-binding; Phosphopantetheine attachment site

  11. XM_036165063.1 → XP_036020956.1  beta-alanine-activating enzyme isoform X12

    Conserved Domains (3) summary
    COG1520
    Location:242 → 474
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    TIGR03300
    Location:411 → 582
    assembly_YfgL; outer membrane assembly lipoprotein YfgL
    pfam00550
    Location:49 → 98
    PP-binding; Phosphopantetheine attachment site

  12. XM_030254391.1 → XP_030110251.1  beta-alanine-activating enzyme isoform X6

    Conserved Domains (4) summary
    COG1520
    Location:592 → 824
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    TIGR03300
    Location:761 → 932
    assembly_YfgL; outer membrane assembly lipoprotein YfgL
    cd17654
    Location:26 → 370
    A_NRPS_acs4; acyl-CoA synthetase family member 4
    pfam00550
    Location:399 → 448
    PP-binding; Phosphopantetheine attachment site

  13. XM_036165064.1 → XP_036020957.1  beta-alanine-activating enzyme isoform X12

    Conserved Domains (3) summary
    COG1520
    Location:242 → 474
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    TIGR03300
    Location:411 → 582
    assembly_YfgL; outer membrane assembly lipoprotein YfgL
    pfam00550
    Location:49 → 98
    PP-binding; Phosphopantetheine attachment site

  14. XM_006534860.4 → XP_006534923.1  beta-alanine-activating enzyme isoform X10

    Conserved Domains (4) summary
    COG1520
    Location:392 → 624
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    TIGR03300
    Location:561 → 732
    assembly_YfgL; outer membrane assembly lipoprotein YfgL
    pfam00550
    Location:199 → 266
    PP-binding; Phosphopantetheine attachment site
    cl17068
    Location:4 → 170
    AFD_class_I; Adenylate forming domain, Class I superfamily

  15. XM_036165062.1 → XP_036020955.1  beta-alanine-activating enzyme isoform X11

    Conserved Domains (4) summary
    COG1520
    Location:392 → 689
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    sd00039
    Location:453 → 487
    7WD40; WD40 repeat [structural motif]
    pfam00550
    Location:199 → 248
    PP-binding; Phosphopantetheine attachment site
    cl17068
    Location:4 → 170
    AFD_class_I; Adenylate forming domain, Class I superfamily

  16. XM_017320841.3 → XP_017176330.1  beta-alanine-activating enzyme isoform X3

    Conserved Domains (3) summary
    COG1520
    Location:754 → 962
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    cd17654
    Location:15 → 532
    A_NRPS_acs4; acyl-CoA synthetase family member 4
    pfam00550
    Location:561 → 628
    PP-binding; Phosphopantetheine attachment site

  17. XM_006534859.5 → XP_006534922.1  beta-alanine-activating enzyme isoform X9

    Conserved Domains (3) summary
    COG1520
    Location:592 → 800
    PQQ; Outer membrane protein assembly factor BamB, contains PQQ-like beta-propeller repeat [Cell wall/membrane/envelope biogenesis]
    cd17654
    Location:26 → 370
    A_NRPS_acs4; acyl-CoA synthetase family member 4
    pfam00550
    Location:399 → 466
    PP-binding; Phosphopantetheine attachment site

RNA

  1. XR_880391.3 RNA Sequence

  2. XR_880390.3 RNA Sequence

  3. XR_389268.4 RNA Sequence

  4. XR_004942502.1 RNA Sequence

  5. XR_004942498.1 RNA Sequence

    Related
    ENSMUST00000126741.8

  6. XR_004942501.1 RNA Sequence

  7. XR_004942499.1 RNA Sequence

  8. XR_001784673.2 RNA Sequence

  9. XR_004942500.1 RNA Sequence

  10. XR_389267.3 RNA Sequence

Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q80WC9.1 GenPept UniProtKB/Swiss-Prot:Q80WC9

Gene LinkOut

The following LinkOut resources are supplied by external providers. These providers are responsible for maintaining the links.

Chemical Information
Molecular Biology Databases
Research Materials
Tools