Page last updated: 2024-10-06
Mitochondrial DNA Depletion Syndrome
Proteins (27)
| Protein | Synonyms | Taxonomy |
|---|---|---|
| Adenosine deaminase | EC 3.5.4.4; Adenosine aminohydrolase | Rattus norvegicus (Norway rat) |
| Guanine deaminase | Guanase; Guanine aminase; EC 3.5.4.3; Guanine aminohydrolase; GAH | Rattus norvegicus (Norway rat) |
| Ectonucleoside triphosphate diphosphohydrolase 8 | E-NTPDase 8; NTPDase 8; NTPDase8; EC 3.6.1.5 | Rattus norvegicus (Norway rat) |
| Inosine-5'-monophosphate dehydrogenase 1 | IMP dehydrogenase 1; IMPD 1; IMPDH 1; EC 1.1.1.205 | Rattus norvegicus (Norway rat) |
| Deoxycytidine kinase | dCK; EC 2.7.1.74; Deoxyadenosine kinase; 2.7.1.76; Deoxyguanosine kinase; 2.7.1.113 | Rattus norvegicus (Norway rat) |
| Thioredoxin | Trx | Rattus norvegicus (Norway rat) |
| Amidophosphoribosyltransferase | ATase; EC 2.4.2.14; Glutamine phosphoribosylpyrophosphate amidotransferase; GPAT | Rattus norvegicus (Norway rat) |
| Ribonucleoside-diphosphate reductase subunit M2 | EC 1.17.4.1; Ribonucleotide reductase small chain; Ribonucleotide reductase small subunit | Rattus norvegicus (Norway rat) |
| Adenine phosphoribosyltransferase | APRT; EC 2.4.2.7 | Rattus norvegicus (Norway rat) |
| Inosine triphosphate pyrophosphatase | ITPase; Inosine triphosphatase; EC 3.6.1.9; Non-canonical purine NTP pyrophosphatase; Non-standard purine NTP pyrophosphatase; Nucleoside-triphosphate diphosphatase; Nucleoside-triphosphate pyrophosphatase; NTPase | Rattus norvegicus (Norway rat) |
| GMP synthase [glutamine-hydrolyzing] | EC 6.3.5.2; GMP synthetase; Glutamine amidotransferase | Rattus norvegicus (Norway rat) |
| GMP reductase 1 | GMPR 1; EC 1.7.1.7; Guanosine 5'-monophosphate oxidoreductase 1; Guanosine monophosphate reductase 1 | Rattus norvegicus (Norway rat) |
| Ectonucleoside triphosphate diphosphohydrolase 5 | NTPDase 5; EC 3.6.1.6; Guanosine-diphosphatase ENTPD5; GDPase ENTPD5; 3.6.1.42; Uridine-diphosphatase ENTPD5; UDPase ENTPD5 | Rattus norvegicus (Norway rat) |
| Nucleoside diphosphate kinase 6 | NDK 6; NDP kinase 6; EC 2.7.4.6; nm23-R6 | Rattus norvegicus (Norway rat) |
| ADP-sugar pyrophosphatase | EC 3.6.1.13; 8-oxo-dGDP phosphatase; 3.6.1.58; Nuclear ATP-synthesis protein NUDIX5; 2.7.7.96; Nucleoside diphosphate-linked moiety X motif 5; Nudix motif 5 | Rattus norvegicus (Norway rat) |
| Multifunctional protein ADE2 | Rattus norvegicus (Norway rat) | |
| Bis(5'-nucleosyl)-tetraphosphatase [asymmetrical] | EC 3.6.1.17; Diadenosine 5',5'''-P1,P4-tetraphosphate asymmetrical hydrolase; Ap4A hydrolase; Ap4Aase; Diadenosine tetraphosphatase; Nucleoside diphosphate-linked moiety X motif 2; Nudix motif 2 | Rattus norvegicus (Norway rat) |
| Adenylosuccinate lyase | Rattus norvegicus (Norway rat) | |
| Guanylate kinase | Rattus norvegicus (Norway rat) | |
| Hypothetical protein LOC290558_predicted | Rattus norvegicus (Norway rat) | |
| PDE10A3 | Rattus norvegicus (Norway rat) | |
| Adenylosuccinate synthetase isozyme 2 | Rattus norvegicus (Norway rat) | |
| Soluble guanlate cyclase alpha 2 subunit | Rattus norvegicus (Norway rat) | |
| Ribonucleoside-diphosphate reductase | Rattus norvegicus (Norway rat) | |
| Trifunctional purine biosynthetic protein adenosine-3 [Includes: Phosphoribosylamine--glycine ligase | Rattus norvegicus (Norway rat) | |
| Ribose-phosphate pyrophosphokinase I-like | Rattus norvegicus (Norway rat) | |
| 5-aminoimidazole-4-carboxamide ribonucleotide formyltransferase/IMP cyclohydrolase | Rattus norvegicus (Norway rat) |
Compounds (38)
| Compound | Description |
|---|---|
| phosphoric acid | concise etchant is 37% H3PO4 |
| Orthophosphate | |
| adenine | A purine base and a fundamental unit of ADENINE NUCLEOTIDES. |
| ammonium hydroxide | The hydroxy salt of ammonium ion. It is formed when AMMONIA reacts with water molecules in solution. |
| Carbon Dioxide | A colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. |
| glutamic acid | A non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM. |
| NADH | |
| Adenosine Triphosphate | An adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter. |
| glutamine | A non-essential amino acid present abundantly throughout the body and is involved in many metabolic processes. It is synthesized from GLUTAMIC ACID and AMMONIA. It is the principal carrier of NITROGEN in the body and is an important energy source for many cells. |
| adenosine diphosphate | Adenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position. |
| adenosine monophosphate | Adenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position. |
| adenosine | A nucleoside that is composed of ADENINE and D-RIBOSE. Adenosine or adenosine derivatives play many important biological roles in addition to being components of DNA and RNA. Adenosine itself is a neurotransmitter. |
| phosphoribosyl pyrophosphate | The key substance in the biosynthesis of histidine, tryptophan, and purine and pyrimidine nucleotides. |
| Hydrogen Peroxide | A 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. |
| Water | A 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) |
| Oxygen | An 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. |
| Adenosine Diphosphate Ribose | An ester formed between the aldehydic carbon of RIBOSE and the terminal phosphate of ADENOSINE DIPHOSPHATE. It is produced by the hydrolysis of nicotinamide-adenine dinucleotide (NAD) by a variety of enzymes, some of which transfer an ADP-ribosyl group to target proteins. |
| phosphoribosyl-N-formylglycineamide | |
| glycineamide ribonucleotide | structure |
| uric acid | An oxidation product, via XANTHINE OXIDASE, of oxypurines such as XANTHINE and HYPOXANTHINE. It is the final oxidation product of purine catabolism in humans and primates, whereas in most other mammals URATE OXIDASE further oxidizes it to ALLANTOIN. |
| 2'-deoxy-5'-adenosine monophosphate | RN given refers to parent cpd |
| 2'-deoxyadenosine | was (MH) DEOXYADENOSINE (68-89) |
| 2'-deoxyadenosine triphosphate | RN given refers to unlabeled parent cpd |
| ribose 1-phosphate | RN given refers to (D)-isomer |
| 2-deoxyribose 1-phosphate | |
| NAD | A 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) |
| xanthosine | |
| xanthosine 5'-triphosphate | RN given refers to parent cpd |
| xanthine | A purine base found in most body tissues and fluids, certain plants, and some urinary calculi. It is an intermediate in the degradation of adenosine monophosphate to uric acid, being formed by oxidation of hypoxanthine. The methylated xanthine compounds caffeine, theobromine, and theophylline and their derivatives are used in medicine for their bronchodilator effects. (Dorland, 28th ed) |
| saicar | adenosylsuccinase catalyzes its conversion to AICAR |
| aminoimidazole ribotide | |
| carboxyaminoimidazole ribotide | reacts with alanosine to form antimetabolite; substrate for EC 4.1.1.21; structure in second source |
| 5-formamidoimidazole-4-carboxamide ribotide | purine precursor |
| creolin | from refined coal tar oils |
| Cyclic AMP | An adenine nucleotide containing one phosphate group which is esterified to both the 3'- and 5'-positions of the sugar moiety. It is a second messenger and a key intracellular regulator, functioning as a mediator of activity for a number of hormones, including epinephrine, glucagon, and ACTH. |
| pyrophosphate | |
| aica ribonucleotide | purine precursor that has antineoplastic activity |
| aminoimidazole carboxamide | An imidazole derivative which is a metabolite of the antineoplastic agents BIC and DIC. By itself, or as the ribonucleotide, it is used as a condensation agent in the preparation of nucleosides and nucleotides. Compounded with orotic acid, it is used to treat liver diseases. |