llustration of biochemical interactions: the carnitine shuttle, fatty acid oxidation, and glycolysis
llustration of biochemical interactions: the carnitine shuttle, fatty acid oxidation, and glycolysis. The mitochondrial membrane is not permeable to long-chain fatty acids. The carnitine shuttle has to be used to transfer long-chain fatty acids to the mitochondrial matrix space. Once in the mitochondrial matrix space, long-chain acylcarnitines are converted back to long-chain acyl-coenzyme A derivatives and free carnitine via CPT2. Free carnitine is released and the acyl-coenzyme A derivatives will enter the beta-oxidation pathway. CPT1: Carnitine-Palmitoyltransferase 1; CPT2: Carnitine-Palmitoyltransferase 2; CAT: Carnitine-/Acylcarnitine-Transferase; LC: Long-chain; TCA: Tricarboxylic acid cycle; PC: Pyruvate Carcoxylase; PDH: Pyruvate Dehydrogenase.
Publication
Inborn Errors of Energy Metabolism Associated with Myopathies. (2010) Anibh M. Das, et al. J Biomed Biotechnol. 2010;2010:340849. Figure: F2.| Organism | Group | Word | Match | Source | NCBI Symbol | NCBI ID |
|---|---|---|---|---|---|---|
| Bos taurus | Mammals | CAC | CAC | ncbigene_synonym | SLC25A20 | 518758 |
| Bos taurus | Mammals | CPT2 | CPT2 | ncbigene_symbol | CPT2 | 504502 |
| Bos taurus | Mammals | PC | PC | ncbigene_symbol | PC | 338471 |
| Bos taurus | Mammals | PC | PC | ncbigene_symbol | PC | 100531427 |
| Bos taurus | Mammals | PC | PC | ncbigene_symbol | PC | 102030714 |
| Bos taurus | Mammals | PC | PC | ncbigene_symbol | PC | 102030715 |
| Bos taurus | Mammals | PC | PC | ncbigene_symbol | PC | 102030716 |
| Bos taurus | Mammals | PC | PC | ncbigene_symbol | PC | 102030897 |
| Bos taurus | Mammals | PC | PC | ncbigene_symbol | PC | 102030942 |
| Bos taurus | Mammals | PC | PC | ncbigene_symbol | PC | 102030982 |
| Bos taurus | Mammals | PC | PC | ncbigene_symbol | PC | 102031061 |
| Sus scrofa | Mammals | acids | ACID | ncbigene_symbol | ACID | 100326236 |
| Sus scrofa | Mammals | acids | ACID | ncbigene_symbol | ACID | 100326244 |
| Sus scrofa | Mammals | CPT1 | CPT1 | ncbigene_synonym | CPT1A | 399527 |
| Sus scrofa | Mammals | CPT1 | CPT1 | ncbigene_synonym | CPT1B | 399528 |
| Sus scrofa | Mammals | CPT2 | CPT2 | ncbigene_symbol | CPT2 | 100519491 |
| Sus scrofa | Mammals | PC | PC | ncbigene_symbol | PC | 397630 |
| Homo sapiens | Primates | CPT1 | CPT1 | ncbigene_synonym | CPT1A | 1374 |
| Homo sapiens | Primates | CPT1 | CPT1 | ncbigene_synonym | CPT2 | 1376 |
| Homo sapiens | Primates | CPT1 | CPT1 | ncbigene_synonym | CHPT1 | 56994 |
| Homo sapiens | Primates | CAC | CAC | ncbigene_synonym | CA2 | 760 |
| Homo sapiens | Primates | CAC | CAC | ncbigene_synonym | SLC25A20 | 788 |
| Homo sapiens | Primates | PDH | PDH | ncbigene_synonym | PDP1 | 54704 |
| Homo sapiens | Primates | PDH | PDH | famplex_relations | DLAT | 1737 |
| Homo sapiens | Primates | PDH | PDH | famplex_relations | DLD | 1738 |
| Homo sapiens | Primates | PDH | PDH | famplex_relations | PDHA1 | 5160 |
| Homo sapiens | Primates | PDH | PDH | famplex_relations | PDHA2 | 5161 |
| Homo sapiens | Primates | PDH | PDH | famplex_relations | PDHB | 5162 |
| Homo sapiens | Primates | PDH | PDH | famplex_relations | PDHX | 8050 |
| Homo sapiens | Primates | PC | PC | ncbigene_symbol | PC | 5091 |
| Drosophila melanogaster | Invertebrates | CoA | COA | ncbigene_symbol | Coa | 248135 |
| Drosophila melanogaster | Invertebrates | CPT1 | CPT1 | ncbigene_synonym | whd | 36109 |
| Drosophila melanogaster | Invertebrates | CAC | CAC | ncbigene_symbol | cac | 32158 |
| Drosophila melanogaster | Invertebrates | CAC | CAC | ncbigene_synonym | cact | 34969 |
| Drosophila melanogaster | Invertebrates | CAC | CAC | ncbigene_synonym | tRNA:His-GTG-1-1 | 3771766 |
| Drosophila melanogaster | Invertebrates | CAC | CAC | ncbigene_synonym | tRNA:His-GTG-1-2 | 3772151 |
| Drosophila melanogaster | Invertebrates | CAC | CAC | ncbigene_synonym | tRNA:His-GTG-1-4 | 3772362 |
| Drosophila melanogaster | Invertebrates | CAC | CAC | ncbigene_synonym | tRNA:His-GTG-1-5 | 3772446 |
| Drosophila melanogaster | Invertebrates | CAC | CAC | ncbigene_synonym | tRNA:His-GTG-1-3 | 3772454 |
| Drosophila melanogaster | Invertebrates | CPT2 | CPT2 | ncbigene_symbol | CPT2 | 38355 |
| Drosophila melanogaster | Invertebrates | PDH | PDH | ncbigene_symbol | Pdh | 39812 |
| Drosophila melanogaster | Invertebrates | PDH | PDH | ncbigene_synonym | Pdha | 31406 |
| Drosophila melanogaster | Invertebrates | PDH | PDH | ncbigene_synonym | 43193 | |
| Drosophila melanogaster | Invertebrates | PDH | PDH | ncbigene_synonym | Pdhb | 43437 |
| Drosophila melanogaster | Invertebrates | PC | PC | ncbigene_symbol | Pc | 40358 |
| Word | Match | MeSH | Name | ChEBI |
|---|---|---|---|---|
| Acyl CoA | NA | mesh:D000214 | ||
| Acyl-CoA | NA | mesh:D000214 | ||
| Carnitine | NA | mesh:D002331 | ||
| Acyl carnitine | NA | mesh:D002331 | ||
| Glucose-6-P | Glucose | mesh:D005947 | D-glucopyranose | chebi:4167 |
| Oxaloacetate | NA | mesh:D010071 | ||
| oxaloacetate | NA | mesh:D010071 | ||
| Pyruvate | NA | mesh:D011773 | ||
| lactate | NA | mesh:D019344 |
Disease mentions
| Word | Match | MeSH | Name | DOID |
|---|