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Tbio
ALDH4A1
Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial

Protein Summary
Description
Irreversible conversion of delta-1-pyrroline-5-carboxylate (P5C), derived either from proline or ornithine, to glutamate. This is a necessary step in the pathway interconnecting the urea and tricarboxylic acid cycles. The preferred substrate is glutamic gamma-semialdehyde, other substrates include succinic, glutaric and adipic semialdehydes. This protein belongs to the aldehyde dehydrogenase family of proteins. This enzyme is a mitochondrial matrix NAD-dependent dehydrogenase which catalyzes the second step of the proline degradation pathway, converting pyrroline-5-carboxylate to glutamate. Deficiency of this enzyme is associated with type II hyperprolinemia, an autosomal recessive disorder characterized by accumulation of delta-1-pyrroline-5-carboxylate (P5C) and proline. Alternatively spliced transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq, Jun 2009]
Uniprot Accession IDs
Gene Name
Ensembl ID
  • ENST00000290597
  • ENSP00000290597
  • ENSG00000159423
  • ENST00000375341
  • ENSP00000364490
  • ENST00000538309
  • ENSP00000442988
  • ENST00000538839
  • ENSP00000446071

Symbol
  • ALDH4
  • P5CDH
  • P5CD
  • ALDH4
  • P5CDh
Illumination Graph
Knowledge Table
Most Knowledge About
Knowledge Value (0 to 1 scale)
protein domain
0.92
disease perturbation
0.79
histone modification site profile
0.78
gene perturbation
0.77
co-expressed gene
0.76


Related Tools
GENEVA
GENEVA (GENe Expression Variance Analysis) allows you to identify RNA-sequencing datasets from the Gene Expression Omnibus (GEO) that contain conditions modulating a gene or a gene signature.
GlyGen
GlyGen is a data integration and dissemination project for carbohydrate and glycoconjugate related data.
ARCHS4
ARCHS4 provides access to gene-function predictions based on RNA-seq co-expression, and gene expression levels across cell and tissues.
IDG Development Level Summary
Tdark

These are targets about which virtually nothing is known. They do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 63.53   (req: < 5)
Gene RIFs: 4   (req: <= 3)
Antibodies: 259   (req: <= 50)
Tbio

These targets do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 63.53   (req: >= 5)
Gene RIFs: 4   (req: > 3)
Antibodies: 259   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 9
Tchem

Target has at least one ChEMBL compound with an activity cutoff of < 30 nM - AND - satisfies the preceding conditions

Active Ligand: 0
Tclin

Target has at least one approved drug - AND - satisfies the preceding conditions

Active Drug: 0
Orthologs (11)
1 – 5 of 11
Species
Name
Source ID
Gene ID
OMA
EggNOG
Inparanoid
Chimp
aldehyde dehydrogenase 4 family member A1
VGNC:60
456575
Macaque
aldehyde dehydrogenase 4 family member A1
714870
Mouse
MGI:2443883
212647
Horse
aldehyde dehydrogenase 4 family member A1
VGNC:15236
100147246
Cow
aldehyde dehydrogenase 4 family member A1
VGNC:25815
100126042
Species
Name
OMA
EggNOG
Inparanoid
Chimp
aldehyde dehydrogenase 4 family member A1
Macaque
aldehyde dehydrogenase 4 family member A1
Mouse
Horse
aldehyde dehydrogenase 4 family member A1
Cow
aldehyde dehydrogenase 4 family member A1
Protein Structure (4 Structures, 1 AlphaFold Model)
RepresentationColor Scheme

Click on a row in the table to change the structure displayed.
More information can be found at RCSB PDB

AF-P30038-F1-model_v1

AlphaFold Structures Developed by DeepMind and EMBL-EBI

1 – 4 of 4
PDB Structure Id
Ligand
Method
Resolution (Å)
Residues
Fraction of Total Protein
Pub Year
Title
PDB Structure Id
Fraction of Total Protein
Resolution
Pub Year
Pathways (16)
Glyoxylate metabolism and glycine degradation (R-HSA-389661)

Click on a row in the table to change the structure displayed.

Items per page:
1 – 4 of 4
Data Source
Name
Explore in Pharos
Explore in Source
Reactome
Glyoxylate metabolism and glycine degradation
Reactome
Metabolism
Reactome
Metabolism of amino acids and derivatives
Reactome
Proline catabolism
Name
Explore in Pharos
Explore in Source
Glyoxylate metabolism and glycine degradation
Metabolism
Metabolism of amino acids and derivatives
Proline catabolism
Protein-Protein Interactions (100)
1 – 10 of 100
TMEM184A
Tbio
Novelty: 0.16408207
p_int: 0.999998993
p_ni: 9.97e-7
p_wrong: 1.1e-8
Score: 0.232
Data Source: BioPlex,STRINGDB
ARRB1
Tbio
Novelty: 0.00262758
p_int: 0.997706757
p_ni: 0.001123693
p_wrong: 0.00116955
Score: 0.331
Data Source: BioPlex,STRINGDB
ALDH18A1
Tbio
Family: Enzyme
Novelty: 0.00301673
Score: 0.99
Data Source: STRINGDB
PRODH2
Tbio
Family: Enzyme
Novelty: 0.09377289
Score: 0.984
Data Source: STRINGDB
OAT
Tchem
Family: Enzyme
Novelty: 0.00125611
Score: 0.983
Data Source: STRINGDB
PYCR1
Tbio
Family: Enzyme
Novelty: 0.00220832
Score: 0.979
Data Source: STRINGDB
GOT1
Tbio
Family: Enzyme
Novelty: 0.00448817
Score: 0.969
Data Source: STRINGDB
GLUD1
Tbio
Family: Enzyme
Novelty: 0.00301109
Score: 0.963
Data Source: STRINGDB
PYCR2
Tbio
Family: Enzyme
Novelty: 0.05660996
Score: 0.956
Data Source: STRINGDB
PYCR3
Tbio
Family: Enzyme
Novelty: 0.1041757
Score: 0.954
Data Source: STRINGDB
Publication Statistics
PubMed Score  63.53

PubMed score by year
PubTator Score  15.09

PubTator score by year
Amino Acid Sequence
Residue Counts
Protein Sequence
ProtVista Viewer