Glutarate (BioCAD00000011698)

blood cerebrospinal fluid (csf) feces saliva urine placenta prostate

Metabolite Card

Formula: C5H8O4 (132.0423)
SMILES: OC(=O)CCCC(O)=O

Synonyms [en]

glutaric acid; glutarate; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Glutarsaeure; Pentandioate

Reviewed

Last reviewed on 2024-06-28.

Cite this Page

Glutarate. 数据之源，洞见之始. SMRUCC genomics institute, a synthetic life researcher from China. https://biocad_registry.innovation.ac.cn/s/(-)-arctiin (retrieved 2026-01-03) (CAD Registry RN: BioCAD00000011698). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

Note

Glutaric acid is a simple five-carbon linear dicarboxylic acid. Glutaric acid is naturally produced in the body during the metabolism of some amino acids, including lysine and tryptophan. Glutaric acid may cause irritation to the skin and eyes. When present in sufficiently high levels, glutaric acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of glutaric acid are associated with at least three inborn errors of metabolism, including glutaric aciduria type I, malonyl-CoA decarboxylase deficiency, and glutaric aciduria type III. Glutaric aciduria type I (glutaric acidemia type I, glutaryl-CoA dehydrogenase deficiency, GA1, or GAT1) is an inherited disorder in which the body is unable to completely break down the amino acids lysine, hydroxylysine, and tryptophan due to a deficiency of mitochondrial glutaryl-CoA dehydrogenase (EC 1.3.99.7, GCDH). Excessive levels of their intermediate breakdown products (e.g. glutaric acid, glutaryl-CoA, 3-hydroxyglutaric acid, glutaconic acid) can accumulate and cause damage to the brain (and also other organs). Babies with glutaric acidemia type I are often born with unusually large heads (macrocephaly). Macrocephaly is amongst the earliest signs of GA1. GA1 also causes secondary carnitine deficiency because glutaric acid, like other organic acids, is detoxified by carnitine. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated glutaric aciduria. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures. Treatment of glutaric aciduria is mainly based on the restriction of lysine intake, supplementation of carnitine, and an intensification of therapy during intercurrent illnesses. The major principle of dietary treatment is to reduce the production of glutaric acid and 3-hydroxyglutaric acid by restriction of natural protein, in general, and of lysine, in particular (PMID: 17465389, 15505398). Glutaric acid has also been found in Escherichia (PMID: 30143200).

Cytoplasm
Entity Information

DBLinks

Other DBLinks
  • CAS Registry Number: 110-94-1
  • CAS Registry Number: 17015-08-6
  • CAS Registry Number: 68603-87-2
  • CAS Registry Number: 68937-69-9
  • PubChem: 3772
  • PubChem: 743
  • ChEBI: ChEBI:17859
  • HMDB: HMDB0000661
  • LipidMaps: LMFA01170046
  • KEGG: C00489
  • NCBI MeSH: glutaric acid
  • Wikipedia: Glutaric_acid
  • DrugBank: DB03553
  • RefMet: RM0155778
  • MoNA: BAF_UVA_POS000594
  • MoNA: BAF_UVA_POS000595
  • MoNA: BAF_UVA_POS000596
  • MoNA: BAF_UVA_POS000597
  • MoNA: BAF_UVA_POS000751
  • MoNA: CCMSLIB00005464009
  • MoNA: CCMSLIB00005464016
  • MoNA: CCMSLIB00005464030
  • MoNA: CCMSLIB00005464042
  • MoNA: CCMSLIB00005720801
  • MoNA: FiehnHILIC001222
  • MoNA: FiehnHILIC002733
  • MoNA: FiehnLib001117
  • MoNA: GLS00138
  • MoNA: HMDB0000661_c_ms_1117
  • MoNA: HMDB0000661_ms_ms_903
  • MoNA: HMDB0000661_ms_ms_904
  • MoNA: HMDB0000661_ms_ms_905
  • MoNA: JP003894
  • MoNA: KO000873
  • MoNA: KO000874
  • MoNA: KO000875
  • MoNA: KO000876
  • MoNA: KO000877
  • MoNA: KZ000031
  • MoNA: KZ000138
  • MoNA: MoNA023878
  • MoNA: MoNA034344
  • MoNA: MoNA034346
  • MoNA: MoNA034348
  • MoNA: MoNA035596
  • MoNA: MoNA035597
  • MoNA: MoNA035600
  • MoNA: MoNA037987
  • MoNA: MoNA038385
  • MoNA: OUF00249
  • MoNA: PR010158
  • MoNA: PR100556
  • MoNA: PS023401
  • MoNA: PS023402
  • MoNA: PS023407
  • MoNA: PT202340
  • Metlin: METLIN_3254
  • Coconut NaturalProduct: CNP0251636.1
  • Coconut NaturalProduct: CNP0256820.0

Class / Ontology

Metabolic Network
ID EC Number Name
KEGG:R02401 1.2.1.20 glutarate-semialdehyde:NAD+ oxidoreductase
KEGG:R02402 6.2.1.6 glutarate:CoA ligase (ADP-forming)
KEGG:R07402 3.5.1.93 (7R)-7-(4-carboxybutanamido)cephalosporanate amidohydrolase
KEGG:R10982 1.2.1.- glutarate-semialdehyde:NADP+ oxidoreductase
KEGG:R12216 1.14.11.64 glutarate,2-oxoglutarate:oxygen oxidoreductase ((S)-2-hydroxyglutarate-forming)
KEGG:R13035 2.8.3.- C00489 + C00091<=>C00527 + C00042
View More
Organism Source

Taxonomy Source

  1. Acanthamoeba culbertsoni [ncbi taxid: 43142]
  2. Acicarpha tribuloides [ncbi taxid: 49581]
  3. Aconitum palmatum [ncbi taxid: 279772]
  4. Aconitum tianschanicum [ncbi taxid: ]
  5. Acronychia acidula [ncbi taxid: 43715]
  6. Aglaia cordata [ncbi taxid: ]
  7. Albatrellus caeruleoporus [ncbi taxid: ]
  8. Alternaria infectoria [ncbi taxid: 45303]
  9. Amanita pseudoporphyria [ncbi taxid: 67725]
  10. Aniba rosaeodora [ncbi taxid: 1352902]
  11. Aplysilla rosea [ncbi taxid: 1609255]
  12. Aplysinella rhax [ncbi taxid: 375147]
  13. Arbacia pustulosa [ncbi taxid: ]
  14. Ariocarpus fissuratus [ncbi taxid: 866924]
  15. Artemisia laciniata [ncbi taxid: 401909]
  16. Beta vulgaris [ncbi taxid: 161934]
  17. Bidens tripartita [ncbi taxid: 51276]
  18. Bos taurus [ncbi taxid: 9913]
  19. Bridelia moonii [ncbi taxid: ]
  20. Bubalus bubalis [ncbi taxid: 89462]
  21. Bupleurum aureum [ncbi taxid: 428561]
  22. Cacalia robusta [ncbi taxid: ]
  23. Callitris macleayana [ncbi taxid: 214231]
  24. Carpesium faberi [ncbi taxid: 2558930]
  25. Castanopsis hystrix [ncbi taxid: 167390]
  26. Centaurea arbutifolia [ncbi taxid: ]
  27. Centaurea musimomum [ncbi taxid: ]
  28. Chlamydomonas allensworthii [ncbi taxid: 51691]
  29. Cladonia pityrea [ncbi taxid: ]
  30. Claviceps microcephala [ncbi taxid: 1294628]
  31. Codonopsis cordifolioidea [ncbi taxid: 1392592]
  32. Corydalis sewerzowi [ncbi taxid: 3396423]
  33. Crematogaster africana [ncbi taxid: 3396915]
  34. Cryptolestes pusillus [ncbi taxid: 1173686]
  35. Dahlstedtia pentaphylla [ncbi taxid: 1645771]
  36. Daphnia magna [ncbi taxid: 35525]
  37. Dickeya chrysanthemi [ncbi taxid: 556]
  38. Erium villosum [ncbi taxid: ]
  39. Erlangea cordifolia [ncbi taxid: ]
  40. Erylus nobilis [ncbi taxid: ]
  41. Eucalyptus citriodora [ncbi taxid: ]
  42. Euclea natalensis [ncbi taxid: 85202]
  43. Euonymus atropurpurea [ncbi taxid: ]
  44. Eupatorium recurvans [ncbi taxid: ]
  45. Euphausia superba [ncbi taxid: 6819]
  46. Flemingia philippinensis [ncbi taxid: 634991]
  47. Gaillardia tontalensis [ncbi taxid: ]
  48. Genista sessilifolia [ncbi taxid: 147456]
  49. Gomphostemma parviflorum [ncbi taxid: 2980166]
  50. Goupia tomentosa [ncbi taxid: ]
  51. Helichrysum zeyheri [ncbi taxid: 630344]
  52. Herbertus aduncus [ncbi taxid: 284511]
  53. Hippobroma longiflora [ncbi taxid: 368676]
  54. Homo sapiens [ncbi taxid: 9606]
  55. Hypericum japonicum [ncbi taxid: 282542]
  56. Lambertella corni [ncbi taxid: ]
  57. Laurencia claviformis [ncbi taxid: ]
  58. Lepidorhinus squamosus [ncbi taxid: ]
  59. Ligusticum lucidum [ncbi taxid: 1389444]
  60. Luvunga scandens [ncbi taxid: 645647]
  61. Machaerium kuhlmannii [ncbi taxid: ]
  62. Michelia longifolia [ncbi taxid: ]
  63. Morus australis [ncbi taxid: 66392]
  64. Mytilus galloprovincialis [ncbi taxid: 29158]
  65. Nephrolepis biserrata [ncbi taxid: 389499]
  66. Onoseris alata [ncbi taxid: 3153322]
  67. Othonna cylindrica [ncbi taxid: ]
  68. Othonna intermedia [ncbi taxid: 3065563]
  69. Pertusaria pseudocorallina [ncbi taxid: 3397973]
  70. Petrosia corticata [ncbi taxid: 3294655]
  71. Picea jezoensis [ncbi taxid: 67778]
  72. Piper acutifolium [ncbi taxid: ]
  73. Pityrodia ternifolia [ncbi taxid: 664408]
  74. Psiadia arabica [ncbi taxid: ]
  75. Psilotum nudum [ncbi taxid: 3240]
  76. Pteris plumbea [ncbi taxid: ]
  77. Rhizopus stolonifer [ncbi taxid: 4846]
  78. Rhodococcus ruber [ncbi taxid: 1830]
  79. Salicornia herbacea [ncbi taxid: ]
  80. Scytonema ocellatum [ncbi taxid: 1246988]
  81. Senecio taiwanensis [ncbi taxid: ]
  82. Senecio zoellneri [ncbi taxid: ]
  83. Sessibugula translucens [ncbi taxid: ]
  84. Sicana odorifera [ncbi taxid: 386230]
  85. Solanum diflorum [ncbi taxid: ]
  86. Sorbus coronata [ncbi taxid: ]
  87. Spiraea formosa [ncbi taxid: ]
  88. Staehelina fruticosa [ncbi taxid: 313546]
  89. Stigmodera macularia [ncbi taxid: 1580771]
  90. Streptomyces argillaceus [ncbi taxid: 41951]
  91. Streptomyces eurocidicus [ncbi taxid: 66423]
  92. Streptomyces fradiae [ncbi taxid: 1906]
  93. Strychnos panamensis [ncbi taxid: 1040910]
  94. Suaeda salsa [ncbi taxid: 126914]
  95. Syringa pubescens subsp. patula [ncbi taxid: 150407]
  96. Tetraploa aristata [ncbi taxid: 682077]
  97. Thalictrum fauriei [ncbi taxid: ]
  98. Trapa bicornis [ncbi taxid: 236974]
  99. Trichoscypha acuminata [ncbi taxid: 289776]
  100. Uraria picta [ncbi taxid: 1034059]
  101. Uroblaniulus canadensis [ncbi taxid: 118482]
  102. Vallaris heynei [ncbi taxid: ]
  103. Vellozia streptophylla [ncbi taxid: 3373689]
  104. Xyris indica [ncbi taxid: 2919641]
  105. Zygosaccharomyces rouxii [ncbi taxid: 4956]

Pathway Synthetic

pathway id name
PathBank:SMP0000811 L-Glutamate Metabolism
WikiPathways:WP143 Fatty acid beta-oxidation
WikiPathways:WP3604 Biochemical pathways: part I
WikiPathways:WP506 Fatty acid beta-oxidation
WikiPathways:WP1307 Fatty acid beta-oxidation
WikiPathways:WP3622 AtMetExpress overview
WikiPathways:WP4519 Cerebral organic acidurias, including diseases
WikiPathways:WP1061 Fatty acid beta-oxidation
PathBank:SMP0121273 L-Glutamate Metabolism
PathBank:SMP0002434 Lysine Metabolism
View All Pathways