79287-9
Carnitine biosynthesis intermediates panel - Urine
Active
14285-1 Carnitine free (C0) [Moles/volume] in Urine
Part Descriptions
LP18431-4 Carnitine.free (C0)
Carnitine.free is a primary marker for carnitine uptake defect, propionic acidemia, maternal carnitine uptake defect, carnitine palmitoyltransferase I deficiency and primary lactic acidemia (various types). It is also a secondary marker for maternal glutaric acidemia type I.
Source: Regenstrief LOINC
Fully-Specified Name
- Component
- Carnitine.free (C0)
- Property
- SCnc
- Time
- Pt
- System
- Urine
- Scale
- Qn
- Method
Basic Attributes
- Class
- CHEM
- Type
- Laboratory
- First Released
- Version 1.0k
- Last Updated
- Version 2.73 (MIN)
- Order vs. Observation
- Both
- Common Test Rank Get Info
- 18413
Member of these Panels
| LOINC | Long Common Name |
|---|---|
| 79287-9 | Carnitine biosynthesis intermediates panel - Urine |
Member of these Groups Get Info
| LOINC Group | Group Name |
|---|---|
| LG44747-0 | Carnitine.free (C0)| |
| LG34746-4 | Carnitine.free (C0)| |
Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| cs-CZ | Czech (Czechia) | Karnitin (C0) volný: |
| de-AT | German (Austria) | Synonyms: Freies Karnitin /U. |
| de-DE | German (Germany) | Carnitin.frei: |
| el-GR | Greek (Greece) | Καρνιτίνη.ελεύθερη (C0): Synonyms: CHEM Pt Qn SCnc Καρνιτίνη Καρνιτίνη.ελεύθερη (C0) Ούρα |
| es-AR | Spanish (Argentina) | carnitina.libre: |
| es-MX | Spanish (Mexico) | Carnitina libre (C0): |
| es-ES | Spanish (Spain) | Carnitina libre: Synonyms: Cuantitativo |
| et-EE | Estonian (Estonia) | Karnitiin.vaba (C0): Synonyms: Aine kontsentratsioon Juhuslik Kvantitatiivne Uriin |
| fr-CA | French (Canada) | Carnitine.libre (C0): |
| fr-FR | French (France) | Carnitine libre (C0): |
| fr-BE | French (Belgium) | Carnitine.libre (C0): |
| it-IT | Italian (Italy) | Carnitina.libera (C0): Synonyms: Carnitina libera (C0) Chimica Concentrazione di Sostanza Punto nel tempo (episodio) |
| ko-KR | Korean (Korea, Republic Of) | 유리카르니틴: |
| nl-NL | Dutch (Netherlands) | carnitine.vrij (C0): |
| pl-PL | Polish (Poland) | Wolna.karnityna (C0): Synonyms: Karnityna wolna (C0) |
| pt-BR | Portuguese (Brazil) | Carnitina.livre (C0): Synonyms: Substance concentration; |
| ru-RU | Russian (Russian Federation) | Карнитин.свободный(С0): Synonyms: Количественный Молярность; |
| tr-TR | Turkish (Turkey) | Karnitin.serb (C0): |
| zh-CN | Chinese (China) | 肉碱.游离型 (C0): Synonyms: 克分子浓度; |
Example Units
| Unit | Source |
|---|---|
| umol/L | Example UCUM Units |
| mcmol/L | REGENSTRIEF |
17867-3 Carnitine free (C0)/Creatinine [Ratio] in Urine
Part Descriptions
LP18431-4 Carnitine.free (C0)
Carnitine.free is a primary marker for carnitine uptake defect, propionic acidemia, maternal carnitine uptake defect, carnitine palmitoyltransferase I deficiency and primary lactic acidemia (various types). It is also a secondary marker for maternal glutaric acidemia type I.
Source: Regenstrief LOINC
LP32035-5 Creatinine
Creatinine or creatine anhydride, is a breakdown product of creatine phosphate in muscle. The loss of water molecule from creatine results in the formation of creatinine. It is transferred to the kidneys by blood plasma, whereupon it is eliminated by glomular filtration and partial tubular excretion. Creatinine is usually produced at a fairly constant rate and measuring its serum level is a simple test. A rise in blood creatinine levels is observed only with marked damage to functioning nephrons; therefore this test is not suitable for detecting early kidney disease. Creatine and creatinine are metabolized in the kidneys, muscle, liver and pancreas.
Copyright Copyright ©2005-2009 Genome Alberta (Reference to original publication: Wishart DS, Knox C, Guo AC, et al. HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 37(Database issue):D603-610.)
Source: Human Metabolome Database, Creatinine
Fully-Specified Name
- Component
- Carnitine.free (C0)/Creatinine
- Property
- Ratio
- Time
- Pt
- System
- Urine
- Scale
- Qn
- Method
Basic Attributes
- Class
- CHEM
- Type
- Laboratory
- First Released
- Version 1.0l
- Last Updated
- Version 2.73 (MIN)
- Order vs. Observation
- Both
- Common Test Rank Get Info
- 7957
Member of these Panels
| LOINC | Long Common Name |
|---|---|
| 79287-9 | Carnitine biosynthesis intermediates panel - Urine |
| 100428-2 | Carnitine free and total and acylcarnitine panel - Urine |
Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| cs-CZ | Czech (Czechia) | Karnitin.volný (C0)/kreatinin: |
| el-GR | Greek (Greece) | Καρνιτίνη.ελεύθερη (C0)/Κρεατινίνη: Synonyms: CHEM Pt Qn Ratio Καρνιτίνη Καρνιτίνη.ελεύθερη (C0) Κρεατινίνη Ούρα |
| es-AR | Spanish (Argentina) | carnitina.libre/creatinina: |
| es-ES | Spanish (Spain) | Carnitina libre/Creatinina: Synonyms: Cuantitativo |
| es-MX | Spanish (Mexico) | Carnitine.libre (C0) / Creatinina: |
| et-EE | Estonian (Estonia) | Karnitiin.vaba (C0)/kreatiniin: Synonyms: Juhuslik Kvantitatiivne Uriin |
| fr-CA | French (Canada) | Carnitine.libre (C0)/Créatinine: |
| fr-FR | French (France) | Carnitine libre (C0)/créatinine: |
| fr-BE | French (Belgium) | Carnitine.libre (C0)/Créatinine: |
| it-IT | Italian (Italy) | Carnitina.libera (C0)/Creatinina: Synonyms: Carnitina libera (C0) Chimica Punto nel tempo (episodio) |
| ko-KR | Korean (Korea, Republic Of) | 유리카르니틴/크레아티닌: |
| nl-NL | Dutch (Netherlands) | carnitine.vrij (C0)/creatinine: Synonyms: kreatinine |
| pl-PL | Polish (Poland) | Wolna.karnityna (C0)/kreatynina: Synonyms: Karnityna wolna (C0) |
| pt-BR | Portuguese (Brazil) | Carnitina.livre (C0)/Creatinina: Synonyms: ; |
| ru-RU | Russian (Russian Federation) | Карнитин.свободный(С0)/Креатинин: Synonyms: Количественный Коэффициент; |
| tr-TR | Turkish (Turkey) | Karnitin.serb (C0)/Kreatinin: |
| uk-UA | Ukrainian (Ukraine) | Карнітин.вільний (С0)/Креатинін: Synonyms: C0; |
| zh-CN | Chinese (China) | 肉碱.游离型 (C0)/肌酐: Synonyms: CR; |
79331-5 Trimethyllysine/Creatinine [Molar ratio] in Urine
Part Descriptions
LP200267-5 Trimethyllysine
Trimethyllysine (N6,N6,N6-Trimethyl-L-lysine) is a methylated derivative of the amino acid lysine. It is a component of histone proteins, a precursor of carnitine and a coenzyme of fatty acid oxidation. N6,N6,N6-Trimethyl-L-lysine residues are found in a number of proteins and are generated by the action of S-adenosyl-L-methionine on exposed lysine residues. When trimethyllysine is released from cognate proteins via proteolysis, it serves as a precursor for carnitine biosynthesis. Mitochondrial 6-N-trimethyllysine dioxygenase converts 6-N-trimethyllysine to 3-hydroxy-6-N-trimethyllysine as the first step for carnitine biosynthesis. Because the subsequent carnitine biosynthesis enzymes are cytosolic, 3-hydroxy-6-N-trimethyllysine must be transported out of the mitochondria by a putative mitochondrial 6-N-trimethyllysine/3-hydroxy-6-N-trimethyllysine transporter system. Plasma -N-trimethyllysine concentrations are significantly lower in systemic carnitine deficiency patients compared to normal individuals, but no significant difference in urinary -N-trimethyllysine excretion is seen between the two groups.[HMDB ID: HMDB01325]
Copyright Copyright ©2005-2009 Genome Alberta (Reference to original publication: Wishart DS, Knox C, Guo AC, et al. HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 37(Database issue):D603-610.)
Source: Human Metabolome Database
LP32035-5 Creatinine
Creatinine or creatine anhydride, is a breakdown product of creatine phosphate in muscle. The loss of water molecule from creatine results in the formation of creatinine. It is transferred to the kidneys by blood plasma, whereupon it is eliminated by glomular filtration and partial tubular excretion. Creatinine is usually produced at a fairly constant rate and measuring its serum level is a simple test. A rise in blood creatinine levels is observed only with marked damage to functioning nephrons; therefore this test is not suitable for detecting early kidney disease. Creatine and creatinine are metabolized in the kidneys, muscle, liver and pancreas.
Copyright Copyright ©2005-2009 Genome Alberta (Reference to original publication: Wishart DS, Knox C, Guo AC, et al. HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 37(Database issue):D603-610.)
Source: Human Metabolome Database, Creatinine
Fully-Specified Name
- Component
- Trimethyllysine/Creatinine
- Property
- SRto
- Time
- Pt
- System
- Urine
- Scale
- Qn
- Method
Basic Attributes
- Class
- CHEM
- Type
- Laboratory
- First Released
- Version 2.54
- Last Updated
- Version 2.54 (ADD)
- Order vs. Observation
- Both
Member of these Panels
| LOINC | Long Common Name |
|---|---|
| 79287-9 | Carnitine biosynthesis intermediates panel - Urine |
Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| cs-CZ | Czech (Czechia) | Trimethyllysin/kreatinin: |
| el-GR | Greek (Greece) | Τριμεθυλολυσίνη/Κρεατινίνη: Synonyms: CHEM Pt Qn SRto Κρεατινίνη Ούρα Τριμεθυλολυσίνη |
| es-ES | Spanish (Spain) | Trimetil lisina/Creatinina: Synonyms: Cuantitativo |
| es-MX | Spanish (Mexico) | Trimetilisina / Creatinina: |
| fr-FR | French (France) | Triméthyllysine/créatinine: |
| it-IT | Italian (Italy) | Trimetil-lisina/Creatinina: Synonyms: Chimica Punto nel tempo (episodio) Rapporto di sostanza |
| nl-NL | Dutch (Netherlands) | trimethyllysine/creatinine: Synonyms: kreatinine |
| pl-PL | Polish (Poland) | Trimetylolizyna/kreatynina: |
| tr-TR | Turkish (Turkey) | Trimetillizin/Kreatinin: Synonyms: SubsOranı |
| zh-CN | Chinese (China) | 三甲基赖氨酸/肌酐: Synonyms: CR; |
Example Units
| Unit | Source |
|---|---|
| umol/mol{creat} | Example UCUM Units |
79311-7 Gamma butyrobetaine/Creatinine [Molar ratio] in Urine
Part Descriptions
LP200429-1 Gamma butyrobetaine
Gamma-butyrobetaine dioxygenase catalyzes the formation of L-carnitine from gamma-butyrobetaine (G-butyrobetaine). G-butyrobetaine levels are altered in various carnitine biosynthesis disorders, including X-linked trimethyllysine hydroxylase epsilon (TMLHE) deficiency. Decreased levels of gamma-butyrobetaine and 3-hydroxy-6-N-trimethyllysine along with increased levels of 6-N-trimethyllysine in plasma and urine were found in a patient with a deletion of exon 2 of the trimethyllysine hydroxylase epsilon (TMLHE) gene. PMID: 22566635
Source: Regenstrief LOINC
LP32035-5 Creatinine
Creatinine or creatine anhydride, is a breakdown product of creatine phosphate in muscle. The loss of water molecule from creatine results in the formation of creatinine. It is transferred to the kidneys by blood plasma, whereupon it is eliminated by glomular filtration and partial tubular excretion. Creatinine is usually produced at a fairly constant rate and measuring its serum level is a simple test. A rise in blood creatinine levels is observed only with marked damage to functioning nephrons; therefore this test is not suitable for detecting early kidney disease. Creatine and creatinine are metabolized in the kidneys, muscle, liver and pancreas.
Copyright Copyright ©2005-2009 Genome Alberta (Reference to original publication: Wishart DS, Knox C, Guo AC, et al. HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 37(Database issue):D603-610.)
Source: Human Metabolome Database, Creatinine
Fully-Specified Name
- Component
- Gamma butyrobetaine/Creatinine
- Property
- SRto
- Time
- Pt
- System
- Urine
- Scale
- Qn
- Method
Basic Attributes
- Class
- CHEM
- Type
- Laboratory
- First Released
- Version 2.54
- Last Updated
- Version 2.54 (ADD)
- Order vs. Observation
- Both
Member of these Panels
| LOINC | Long Common Name |
|---|---|
| 79287-9 | Carnitine biosynthesis intermediates panel - Urine |
Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| cs-CZ | Czech (Czechia) | Gama-butyrobetain/kreatinin: |
| el-GR | Greek (Greece) | γ-βουτυροβεταΐνη/Κρεατινίνη: Synonyms: CHEM Pt Qn SRto γ-βουτυροβεταΐνη Κρεατινίνη Ούρα |
| es-ES | Spanish (Spain) | Gamma butirobetaina/Creatinina: Synonyms: Cuantitativo |
| es-MX | Spanish (Mexico) | Gamma butirobetaína / creatinina: |
| fr-FR | French (France) | Gamma butyrobétaïne/créatinine: |
| it-IT | Italian (Italy) | Gsmma butirro-betaina/Creatinina: Synonyms: Chimica Punto nel tempo (episodio) Rapporto di sostanza |
| nl-NL | Dutch (Netherlands) | gamma butyrobetaïne/creatinine: Synonyms: kreatinine |
| pl-PL | Polish (Poland) | Gamma butyrobetaina/kreatynina: |
| tr-TR | Turkish (Turkey) | Gamma butirobetain/Kreatinin: Synonyms: SubsOranı |
| zh-CN | Chinese (China) | Gamma 丁酰甜菜碱/肌酐: Synonyms: CR; |
Example Units
| Unit | Source |
|---|---|
| umol/mmol{creat} | Example UCUM Units |
79313-3 Gamma butyrobetaine/Trimethyllysine [Molar ratio] in Urine
Part Descriptions
LP200429-1 Gamma butyrobetaine
Gamma-butyrobetaine dioxygenase catalyzes the formation of L-carnitine from gamma-butyrobetaine (G-butyrobetaine). G-butyrobetaine levels are altered in various carnitine biosynthesis disorders, including X-linked trimethyllysine hydroxylase epsilon (TMLHE) deficiency. Decreased levels of gamma-butyrobetaine and 3-hydroxy-6-N-trimethyllysine along with increased levels of 6-N-trimethyllysine in plasma and urine were found in a patient with a deletion of exon 2 of the trimethyllysine hydroxylase epsilon (TMLHE) gene. PMID: 22566635
Source: Regenstrief LOINC
LP200451-5 Trimethyllysine
Trimethyllysine (N6,N6,N6-Trimethyl-L-lysine) is a methylated derivative of the amino acid lysine. It is a component of histone proteins, a precursor of carnitine and a coenzyme of fatty acid oxidation. N6,N6,N6-Trimethyl-L-lysine residues are found in a number of proteins and are generated by the action of S-adenosyl-L-methionine on exposed lysine residues. When trimethyllysine is released from cognate proteins via proteolysis, it serves as a precursor for carnitine biosynthesis. Mitochondrial 6-N-trimethyllysine dioxygenase converts 6-N-trimethyllysine to 3-hydroxy-6-N-trimethyllysine as the first step for carnitine biosynthesis. Because the subsequent carnitine biosynthesis enzymes are cytosolic, 3-hydroxy-6-N-trimethyllysine must be transported out of the mitochondria by a putative mitochondrial 6-N-trimethyllysine/3-hydroxy-6-N-trimethyllysine transporter system. Plasma -N-trimethyllysine concentrations are significantly lower in systemic carnitine deficiency patients compared to normal individuals, but no significant difference in urinary -N-trimethyllysine excretion is seen between the two groups.[HMDB ID: HMDB01325]
Copyright Copyright ©2005-2009 Genome Alberta (Reference to original publication: Wishart DS, Knox C, Guo AC, et al. HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 37(Database issue):D603-610.)
Source: Human Metabolome Database
Fully-Specified Name
- Component
- Gamma butyrobetaine/Trimethyllysine
- Property
- SRto
- Time
- Pt
- System
- Urine
- Scale
- Qn
- Method
Basic Attributes
- Class
- CHEM
- Type
- Laboratory
- First Released
- Version 2.54
- Last Updated
- Version 2.54 (ADD)
- Order vs. Observation
- Both
Member of these Panels
| LOINC | Long Common Name |
|---|---|
| 79287-9 | Carnitine biosynthesis intermediates panel - Urine |
Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| cs-CZ | Czech (Czechia) | Gama-butyrobetain/trimethyllysin: |
| el-GR | Greek (Greece) | γ-βουτυροβεταΐνη/Τριμεθυλολυσίνη: Synonyms: CHEM Pt Qn SRto γ-βουτυροβεταΐνη Ούρα Τριμεθυλολυσίνη |
| es-ES | Spanish (Spain) | Gamma butirobetaina/Trimetil lisina: Synonyms: Cuantitativo |
| es-MX | Spanish (Mexico) | Gamma butirobetaína / trimetilisina: |
| fr-FR | French (France) | Gamma butyrobétaïne/triméthyllysine: |
| it-IT | Italian (Italy) | Gsmma butirro-betaina/Trimetil-lisina: Synonyms: Chimica Punto nel tempo (episodio) Rapporto di sostanza |
| nl-NL | Dutch (Netherlands) | gamma butyrobetaïne/trimethyllysine: |
| pl-PL | Polish (Poland) | Gamma butyrobetaina/trimetylolizyna: |
| tr-TR | Turkish (Turkey) | Gamma butirobetain/Trimetillizin: Synonyms: SubsOranı |
| zh-CN | Chinese (China) | Gamma 丁酰甜菜碱/三甲基赖氨酸: Synonyms: Gamma-丁酰甜菜碱; |
Example Units
| Unit | Source |
|---|---|
| {ratio} | Example UCUM Units |
79271-3 3-Hydroxytrimethyl-L-lysine/Creatinine [Molar ratio] in Urine
Part Descriptions
LP200265-9 3-Hydroxytrimethyl-L-lysine
3-Hydroxytrimethyl-L-lysine is found within the Lysine degradation (aka L-carnitine biosynthesis) pathway; it is a byproduct of trimethyllysine dioxygenase (trimethyllysine hydroxylase, EC 1.14.11.8), which converts trimethyllysine + 2-oxoglutarate to 3-Hydroxytrimethyl-L-lysine + succinate. Patients with X-linked trimethyllysine hydroxylase (TMLHE) deficiency, a carnitine deficiency disorder associated with autism, have lower than expected levels of carnitine as well as 3-Hydroxytrimethyl-L-lysine, and elevated levels of trimethyllysine. [OMIM: 300777]
Source: Regenstrief LOINC
LP32035-5 Creatinine
Creatinine or creatine anhydride, is a breakdown product of creatine phosphate in muscle. The loss of water molecule from creatine results in the formation of creatinine. It is transferred to the kidneys by blood plasma, whereupon it is eliminated by glomular filtration and partial tubular excretion. Creatinine is usually produced at a fairly constant rate and measuring its serum level is a simple test. A rise in blood creatinine levels is observed only with marked damage to functioning nephrons; therefore this test is not suitable for detecting early kidney disease. Creatine and creatinine are metabolized in the kidneys, muscle, liver and pancreas.
Copyright Copyright ©2005-2009 Genome Alberta (Reference to original publication: Wishart DS, Knox C, Guo AC, et al. HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 37(Database issue):D603-610.)
Source: Human Metabolome Database, Creatinine
Fully-Specified Name
- Component
- 3-Hydroxytrimethyl-L-lysine/Creatinine
- Property
- SRto
- Time
- Pt
- System
- Urine
- Scale
- Qn
- Method
Basic Attributes
- Class
- CHEM
- Type
- Laboratory
- First Released
- Version 2.54
- Last Updated
- Version 2.54 (MIN)
- Order vs. Observation
- Both
Member of these Panels
| LOINC | Long Common Name |
|---|---|
| 79287-9 | Carnitine biosynthesis intermediates panel - Urine |
Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| cs-CZ | Czech (Czechia) | 3-hydroxytrimethyl-L-lysin/kreatinin: |
| el-GR | Greek (Greece) | 3-Υδροξυτριμεθυλο-L-λυσίνη/Κρεατινίνη: Synonyms: 3-Υδροξυτριμεθυλο-L-λυσίνη CHEM Pt Qn SRto Κρεατινίνη Λυσίνη Ούρα |
| es-ES | Spanish (Spain) | 3-Hidroxi-trimetil-lisina/Creatinina: Synonyms: Cuantitativo |
| es-MX | Spanish (Mexico) | 3-hidroxitrimetil-L-lisina / creatinina: |
| fr-FR | French (France) | 3-hydroxytriméthyl-L-lysine/créatinine: |
| it-IT | Italian (Italy) | 3-Idrossitrimetil-L-lisina/Creatinina: Synonyms: Chimica Punto nel tempo (episodio) Rapporto di sostanza |
| nl-NL | Dutch (Netherlands) | 3-hydroxytrimethyl-L-lysine/creatinine: Synonyms: kreatinine |
| pl-PL | Polish (Poland) | 3-Hydroksytrimetylo-L-lizyna/kreatynina: |
| tr-TR | Turkish (Turkey) | 3-Hidroksitrimetil-L-lizin/Kreatinin: Synonyms: SubsOranı |
| zh-CN | Chinese (China) | 3-羟基三甲基-L-赖氨酸/肌酐: Synonyms: 2,6-二氨基已酸; |
Example Units
| Unit | Source |
|---|---|
| umol/mmol{creat} | Example UCUM Units |
79275-4 3-Hydroxytrimethyl-L-lysine/Trimethyllysine [Molar ratio] in Urine
Part Descriptions
LP200265-9 3-Hydroxytrimethyl-L-lysine
3-Hydroxytrimethyl-L-lysine is found within the Lysine degradation (aka L-carnitine biosynthesis) pathway; it is a byproduct of trimethyllysine dioxygenase (trimethyllysine hydroxylase, EC 1.14.11.8), which converts trimethyllysine + 2-oxoglutarate to 3-Hydroxytrimethyl-L-lysine + succinate. Patients with X-linked trimethyllysine hydroxylase (TMLHE) deficiency, a carnitine deficiency disorder associated with autism, have lower than expected levels of carnitine as well as 3-Hydroxytrimethyl-L-lysine, and elevated levels of trimethyllysine. [OMIM: 300777]
Source: Regenstrief LOINC
LP200451-5 Trimethyllysine
Trimethyllysine (N6,N6,N6-Trimethyl-L-lysine) is a methylated derivative of the amino acid lysine. It is a component of histone proteins, a precursor of carnitine and a coenzyme of fatty acid oxidation. N6,N6,N6-Trimethyl-L-lysine residues are found in a number of proteins and are generated by the action of S-adenosyl-L-methionine on exposed lysine residues. When trimethyllysine is released from cognate proteins via proteolysis, it serves as a precursor for carnitine biosynthesis. Mitochondrial 6-N-trimethyllysine dioxygenase converts 6-N-trimethyllysine to 3-hydroxy-6-N-trimethyllysine as the first step for carnitine biosynthesis. Because the subsequent carnitine biosynthesis enzymes are cytosolic, 3-hydroxy-6-N-trimethyllysine must be transported out of the mitochondria by a putative mitochondrial 6-N-trimethyllysine/3-hydroxy-6-N-trimethyllysine transporter system. Plasma -N-trimethyllysine concentrations are significantly lower in systemic carnitine deficiency patients compared to normal individuals, but no significant difference in urinary -N-trimethyllysine excretion is seen between the two groups.[HMDB ID: HMDB01325]
Copyright Copyright ©2005-2009 Genome Alberta (Reference to original publication: Wishart DS, Knox C, Guo AC, et al. HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 37(Database issue):D603-610.)
Source: Human Metabolome Database
Fully-Specified Name
- Component
- 3-Hydroxytrimethyl-L-lysine/Trimethyllysine
- Property
- SRto
- Time
- Pt
- System
- Urine
- Scale
- Qn
- Method
Basic Attributes
- Class
- CHEM
- Type
- Laboratory
- First Released
- Version 2.54
- Last Updated
- Version 2.54 (MIN)
- Order vs. Observation
- Both
Member of these Panels
| LOINC | Long Common Name |
|---|---|
| 79287-9 | Carnitine biosynthesis intermediates panel - Urine |
Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| cs-CZ | Czech (Czechia) | 3-hydroxytrimethyl-L-lysin/trimethyllysin: |
| el-GR | Greek (Greece) | 3-Υδροξυτριμεθυλο-L-λυσίνη/Τριμεθυλολυσίνη: Synonyms: 3-Υδροξυτριμεθυλο-L-λυσίνη CHEM Pt Qn SRto Λυσίνη Ούρα Τριμεθυλολυσίνη |
| es-ES | Spanish (Spain) | 3-Hidroxi-trimetil-lisina/Trimetil lisina: Synonyms: Cuantitativo |
| es-MX | Spanish (Mexico) | 3-hidroxitrimetil-L-lisina / trimetilisina: |
| fr-FR | French (France) | 3-hydroxytriméthyl-L-lysine/triméthyllysine: |
| it-IT | Italian (Italy) | 3-Idrossitrimetil-L-lisina/Trimetil-lisina: Synonyms: Chimica Punto nel tempo (episodio) Rapporto di sostanza |
| nl-NL | Dutch (Netherlands) | 3-hydroxytrimethyl-L-lysine/trimethyllysine: |
| pl-PL | Polish (Poland) | 3-Hydroksytrimetylo-L-lizyna/trimetylolizyna: |
| tr-TR | Turkish (Turkey) | 3-Hidroksitrimetil-L-lizin/Trimetillizin: Synonyms: SubsOranı |
| zh-CN | Chinese (China) | 3-羟基三甲基-L-赖氨酸/三甲基赖氨酸: Synonyms: 2,6-二氨基已酸; |
Example Units
| Unit | Source |
|---|---|
| {ratio} | Example UCUM Units |
79342-2 Carnitine biosynthesis intermediates pattern [Interpretation] in Urine Narrative
Term Description
Narrative interpretation of carnitine biosynthesis findings in urine. The suspected type of carnitine biosynthesis disorder (e.g. X-linked trimethyllysine hydroxylase deficiency) is reported based on the results.
Fully-Specified Name
- Component
- Carnitine biosynthesis intermediates pattern
- Property
- Imp
- Time
- Pt
- System
- Urine
- Scale
- Nar
- Method
Basic Attributes
- Class
- CHEM
- Type
- Laboratory
- First Released
- Version 2.54
- Last Updated
- Version 2.54 (ADD)
- Order vs. Observation
- Observation
Member of these Panels
| LOINC | Long Common Name |
|---|---|
| 79287-9 | Carnitine biosynthesis intermediates panel - Urine |
Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| cs-CZ | Czech (Czechia) | Karnitin meziprodukty biosyntézy profil: |
| el-GR | Greek (Greece) | Μοτίβο ενδιάμεσων προϊόντων βιοσύνθεσης καρνιτίνης: Synonyms: CHEM Imp Nar Pt Καρνιτίνη Μοτίβο ενδιάμεσων προϊόντων βιοσύνθεσης καρνιτίνης Ούρα |
| es-ES | Spanish (Spain) | Patrón de acilcarnitinas: |
| es-MX | Spanish (Mexico) | Patrón de intermedios de biosíntesis de carnitina: |
| fr-FR | French (France) | Carnitine intermédiaires de synthèse Profil: |
| it-IT | Italian (Italy) | Intermediari nella biosintesi della carnitina, profilo: Synonyms: Chimica Impressione/interpretazione di studio Profilo di intermediari nella biosintesi della car Punto nel tempo (episodio) |
| nl-NL | Dutch (Netherlands) | carnitine biosynthese intermediairen patroon: |
| pl-PL | Polish (Poland) | Wzorzec pośrednich produktów biosyntezy karnityny: |
| tr-TR | Turkish (Turkey) | Karnitin byiosentezi arameabolitleri örüntüsü: Synonyms: Karnitin byiosentezi arameabolitleri paterni |
| zh-CN | Chinese (China) | 肉碱生物合成中间产物模式: Synonyms: 化学; |