Page last updated: 2024-10-06

Cystinosis, Ocular Nonnephropathic

Proteins (9)

ProteinSynonymsTaxonomy
Cystathionine gamma-lyaseEC 4.4.1.1; Cysteine-protein sulfhydrase; Gamma-cystathionaseHomo sapiens (human)
CystinosinHomo sapiens (human)
L-lactate dehydrogenase A chainLDH-A; EC 1.1.1.27; Cell proliferation-inducing gene 19 protein; LDH muscle subunit; LDH-M; Renal carcinoma antigen NY-REN-59Homo sapiens (human)
Glutamate--cysteine ligase regulatory subunitGCS light chain; Gamma-ECS regulatory subunit; Gamma-glutamylcysteine synthetase regulatory subunit; Glutamate--cysteine ligase modifier subunitHomo sapiens (human)
Aspartate aminotransferase, cytoplasmiccAspAT; EC 2.6.1.1; EC 2.6.1.3; Cysteine aminotransferase, cytoplasmic; Cysteine transaminase, cytoplasmic; cCAT; Glutamate oxaloacetate transaminase 1; Transaminase AHomo sapiens (human)
Glutamate--cysteine ligase catalytic subunitEC 6.3.2.2; GCS heavy chain; Gamma-ECS; Gamma-glutamylcysteine synthetaseHomo sapiens (human)
Cysteine dioxygenase type 1EC 1.13.11.20; Cysteine dioxygenase type I; CDO; CDO-IHomo sapiens (human)
Cysteine--tRNA ligase, cytoplasmicEC 6.1.1.16; Cysteinyl-tRNA synthetase; CysRSHomo sapiens (human)
3-mercaptopyruvate sulfurtransferaseMST; EC 2.8.1.2Homo sapiens (human)

Compounds (21)

CompoundDescription
phosphoric acidconcise etchant is 37% H3PO4
pyruvic acidAn intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed)
ammonium hydroxideThe hydroxy salt of ammonium ion. It is formed when AMMONIA reacts with water molecules in solution.
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
cysteineA thiol-containing non-essential amino acid that is oxidized to form CYSTINE.
Adenosine TriphosphateAn adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.
adenosine diphosphateAdenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.
adenosine monophosphateAdenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position.
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.
sulfitesInorganic salts of sulfurous acid.
gamma-glutamylcysteine
beta-mercaptolactatestructure
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)
3-mercaptopyruvic acidRN given refers to parent cpd; structure
cyanidesInorganic salts of HYDROGEN CYANIDE containing the -CN radical. The concept also includes isocyanides. It is distinguished from NITRILES, which denotes organic compounds containing the -CN radical.
pyrophosphate
thiocyanateRN given refers to parent cpd
Slc7a2 protein, mouseRefSeq NM_007514