Page last updated: 2024-10-06

Hyperlysinemia I, Familial

Proteins (13)

ProteinSynonymsTaxonomy
Glutaryl-CoA dehydrogenase, mitochondrialGCD; EC 1.3.8.6Homo sapiens (human)
Enoyl-CoA hydratase, mitochondrialEC 4.2.1.17; Enoyl-CoA hydratase 1; Short-chain enoyl-CoA hydratase; SCEHHomo sapiens (human)
Kynurenine/alpha-aminoadipate aminotransferase, mitochondrialKAT/AadAT; 2-aminoadipate aminotransferase; 2-aminoadipate transaminase; 2.6.1.39; Alpha-aminoadipate aminotransferase; AadAT; Kynurenine aminotransferase II; Kynurenine--oxoglutarate aminotransferase II; Kynurenine--oxoglutarate transaminase 2; 2.6.1.7; Homo sapiens (human)
Peroxisomal sarcosine oxidasePSO; EC 1.5.3.1; EC 1.5.3.7; L-pipecolate oxidase; L-pipecolic acid oxidaseHomo sapiens (human)
Acetyl-CoA acetyltransferase, mitochondrialEC 2.3.1.9; Acetoacetyl-CoA thiolase; T2Homo sapiens (human)
Dihydrolipoyl dehydrogenase, mitochondrialEC 1.8.1.4; Dihydrolipoamide dehydrogenase; Glycine cleavage system L proteinHomo sapiens (human)
Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrialEC 1.2.4.2; Dehydrogenase E1 and transketolase domain-containing protein 1Homo sapiens (human)
Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrialEC 2.3.1.61; 2-oxoglutarate dehydrogenase complex component E2; OGDC-E2; Dihydrolipoamide succinyltransferase component of 2-oxoglutarate dehydrogenase complex; E2KHomo sapiens (human)
Alpha-aminoadipic semialdehyde dehydrogenaseAlpha-AASA dehydrogenase; EC 1.2.1.31; Aldehyde dehydrogenase family 7 member A1; 1.2.1.3; Antiquitin-1; Betaine aldehyde dehydrogenase; 1.2.1.8; Delta1-piperideine-6-carboxylate dehydrogenase; P6c dehydrogenaseHomo sapiens (human)
Cationic amino acid transporter 2CAT-2; CAT2; Low affinity cationic amino acid transporter 2; Solute carrier family 7 member 2Homo sapiens (human)
Hydroxyacyl-coenzyme A dehydrogenase, mitochondrialHCDH; EC 1.1.1.35; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; Short-chain 3-hydroxyacyl-CoA dehydrogenaseHomo sapiens (human)
Mitochondrial ornithine transporter 2Solute carrier family 25 member 2Homo sapiens (human)
Alpha-aminoadipic semialdehyde synthase, mitochondrialLKR/SDHHomo sapiens (human)

Compounds (20)

CompoundDescription
hydronium ion
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
glutamic acidA non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM.
NADH
lysineAn essential amino acid. It is often added to animal feed.
Hydrogen PeroxideA strong oxidizing agent used in aqueous solution as a ripening agent, bleach, and topical anti-infective. It is relatively unstable and solutions deteriorate over time unless stabilized by the addition of acetanilide or similar organic materials.
WaterA clear, odorless, tasteless liquid that is essential for most animal and plant life and is an excellent solvent for many substances. The chemical formula is hydrogen oxide (H2O). (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
OxygenAn element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration.
Coenzyme A
glutaryl-coenzyme A
saccharopinelysine precursor in the aminoadipic acid-lysine pathway in yeast; minor descriptor (75-85); on-line & Index Medicus search LYSINE/analogs & derivatives (75-85); RN given refers to (L)-isomer
allysinestructure
pipecolic acidRN given refers to cpd without isomeric designation
2-aminoadipic acidA metabolite in the principal biochemical pathway of lysine. It antagonizes neuroexcitatory activity modulated by the glutamate receptor, N-METHYL-D-ASPARTATE; (NMDA).
alpha-ketoglutaric acid
NADA coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)
alpha-ketoadipic acid
delta-1-piperidine-6-carboxylic acidintermediate in Pseudomonas lysine metabolism; structure
acetyl coenzyme aAcetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent.
creolinfrom refined coal tar oils