Version 2.80

78360-5 Acyl CoA dehydrogenases [Enzymatic activity/mass] in Fibroblast by [9,10-3H] myristate substrate

Term Description

One method for diagnosing acyl CoA dehydrogenase deficiencies is fatty acid oxidation analysis in cultured fibroblasts. Using multiple fatty acid substrates with varying carbon chain lengths, such as palmitate (16 carbons) [LOINC: 78358-9] and and myristate (14 carbons) (this term), can help differentiate between disorders such as long-chain acyl CoA dehydrogenase deficiency and medium-chain acyl CoA dehydrogenase deficiency. PMID: 2109149 Enzyme activity is measured as nanomoles of fatty acid oxidized per hour per milligram of cell protein (nmol FA/h/mg protein). PMID: 12840153

Fully-Specified Name

Component
Acyl CoA dehydrogenases
Property
CCnt
Time
Pt
System
Fibroblasts
Scale
Qn
Method
[9,10-3H] myristate substrate

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
74533-1 Fatty acid oxidation panel - Fibroblast

Member of these Groups Get Info

LOINC Group Group Name
LG30219-6 Acyl CoA dehydrogenases|CCnt|Pt|Fibroblasts

Language Variants Get Info

Tag Language Translation
el-GR Greek (Greece) Ακυλο-CoA αφυδρογονάσες:CCnt:Pt:Ινοβλάστες:Qn:[9,10-3H] μυριστικό υπόστρωμα
Synonyms: CCnt Ακυλο-CoA αφυδρογονάσες
es-ES Spanish (Spain) Acil Coenzima A deshidrogenasas:Contenido catalítico:Punto temporal:Fibroblastos:Qn:Sustrato [9,10-3H] miristato
Synonyms: Cuantitativo
es-MX Spanish (Mexico) Acil CoA deshidrogenasas:Contenido catalítico:Punto temporal:Fibroblasto:Cuantitativo:[9,10-3H] sustrato de miristato
fr-FR French (France) Acyl CoA déhydrogénases:Catalytique/Masse:Ponctuel:Fibroblastes:Numérique:Substrat [9,10-3H] myristate
it-IT Italian (Italy) Acil CoA deidrogenasi:CCnt:Pt:Fibroblasti:Qn:[9,10-3H] substrato miristato
Synonyms: Chimica Contenuto Catalitico Fibroblasto Punto nel tempo (episodio)
nl-NL Dutch (Netherlands) acyl-CoA-dehydrogenase:enzymatische activiteit/massa:moment:fibroblast:kwantitatief:[9,10-3h] myristaatsubstraat
zh-CN Chinese (China) 酰基 CoA 脱氢酶:催化含量:时间点:成纤维细胞:定量型:[9,10-3H] 肉豆蔻酸底物法
Synonyms: AcdA;酰基辅酶 A 脱氢酶 催化含量(单位质量);单位质量的酶单位数 化学;化学检验项目;化学检验项目类;化学类;化学试验;非刺激耐受型化学检验项目;非刺激耐受型化学检验项目类;非刺激耐受型化学试验;非刺激耐受型化学试验类 可用数量表示的;定量性;数值型;数量型;连续数值型标尺 时刻;随机;随意;瞬间 纤维原细胞;纤维母细胞

Example Units

Unit Source
nmol/h/mg{protein} Example UCUM Units

78358-9 Acyl CoA dehydrogenases [Enzymatic activity/mass] in Fibroblast by [9,10-3H] palmitate substrate

Term Description

Fatty acid oxidation in medium- or long-chain acyl CoA dehydrogenase deficiencies can be measured by quantifying the release of 3H2O from (9,10-3H) palmitate (this term) and myristate [LOINC: 78360-5] substrates added to cultured fibroblast. PMID: 2109149 Fatty acid (i.e. palmitate or myristate) oxidation rates by fibroblasts are expressed as nanomole of 3H fatty acid (3H FA) oxidized per hour per milligram of cell protein (nmol 3H FA/h/mg protein). PMID: 12840153

Fully-Specified Name

Component
Acyl CoA dehydrogenases
Property
CCnt
Time
Pt
System
Fibroblasts
Scale
Qn
Method
[9,10-3H] palmitate substrate

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
74533-1 Fatty acid oxidation panel - Fibroblast

Member of these Groups Get Info

LOINC Group Group Name
LG30219-6 Acyl CoA dehydrogenases|CCnt|Pt|Fibroblasts

Language Variants Get Info

Tag Language Translation
el-GR Greek (Greece) Ακυλο-CoA αφυδρογονάσες:CCnt:Pt:Ινοβλάστες:Qn:[9,10-3H] παλμιτικό υπόστρωμα
Synonyms: CCnt Ακυλο-CoA αφυδρογονάσες
es-ES Spanish (Spain) Acil Coenzima A deshidrogenasas:Contenido catalítico:Punto temporal:Fibroblastos:Qn:Sustrato [9,10-3H] palmitato
Synonyms: Cuantitativo
es-MX Spanish (Mexico) Acil CoA deshidrogenasas:Contenido catalítico:Punto temporal:Fibroblasto:Cuantitativo:Sustrato de palmitato de [9,10-3H]
fr-FR French (France) Acyl CoA déhydrogénases:Catalytique/Masse:Ponctuel:Fibroblastes:Numérique:Substrat [9,10-3H] palmitate
it-IT Italian (Italy) Acil CoA deidrogenasi:CCnt:Pt:Fibroblasti:Qn:[9,10-3H] substrato palmitato
Synonyms: Chimica Contenuto Catalitico Fibroblasto Punto nel tempo (episodio)
nl-NL Dutch (Netherlands) acyl-CoA-dehydrogenase:enzymatische activiteit/massa:moment:fibroblast:kwantitatief:[9,10-3h] myristaatsubstraat
zh-CN Chinese (China) 酰基 CoA 脱氢酶:催化含量:时间点:成纤维细胞:定量型:[9,10-3H] 棕榈酸底物法
Synonyms: AcdA;酰基辅酶 A 脱氢酶 催化含量(单位质量);单位质量的酶单位数 化学;化学检验项目;化学检验项目类;化学类;化学试验;非刺激耐受型化学检验项目;非刺激耐受型化学检验项目类;非刺激耐受型化学试验;非刺激耐受型化学试验类 可用数量表示的;定量性;数值型;数量型;连续数值型标尺 时刻;随机;随意;瞬间 纤维原细胞;纤维母细胞

Example Units

Unit Source
nmol/h/mg{protein} Example UCUM Units

78359-7 Acyl CoA dehydrogenases.palmitate substrate/Acyl CoA dehydrogenases.myristate substrate [Enzymatic activity ratio] in Fibroblast

Term Description

Medium- and long-chain acyl CoA dehydrogenase deficiencies can be differentiated based on the ratio of palmitate oxidation [LOINC: 78358-9] and myristate oxidation [LOINC: 78360-5] in cultured fibroblasts. Patients with medium chain acyl-CoA dehydrogenase deficiency (MCAD) typically have a high palmitate/myristate (P/M) ratio. Long-chain defects, including VLCAD and LCHAD, generally have a low P/M ratio. PMID: 10709659

Fully-Specified Name

Component
Acyl CoA dehydrogenases.palmitate substrate/Acyl CoA dehydrogenases.myristate substrate
Property
CRto
Time
Pt
System
Fibroblasts
Scale
Qn
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
74533-1 Fatty acid oxidation panel - Fibroblast

Language Variants Get Info

Tag Language Translation
el-GR Greek (Greece) Ακυλο-CoA αφυδρογονάσες.παλμιτικό υπόστρωμα/Ακυλο-CoA αφυδρογονάσες.μυριστικό υπόστρωμα:CRto:Pt:Ινοβλάστες:Qn:
Synonyms: CRto Ακυλο-CoA αφυδρογονάσες Ακυλο-CoA αφυδρογονάσες.παλμιτικό υπόστρωμα Μυριστικό Παλμιτικό
es-MX Spanish (Mexico) Acil CoA deshidrogenasas, sustrato palmitato / acil CoA deshidrogenasas, sustrato miristato:Relación catalítica:Punto temporal:Fibroblasto:Cuantitativo:
es-ES Spanish (Spain) Acil Coenzima A deshidrogenasas.sustrato palmitato/Acil Coenzima A deshidrogenasas.sustrato miristato:Relacion de concentración catalítica:Punto temporal:Fibroblastos:Qn:
Synonyms: Cuantitativo
fr-FR French (France) Acyl CoA déhydrogénases substrat palmitate/Acyl CoA déhydrogénases substrat myristate:Catalytique ratio:Ponctuel:Fibroblastes:Numérique:
it-IT Italian (Italy) Acil Co deidrogenasi.substrato palmitato/Acil CoA deidrogenasi.subastrato miristato:CCrto:Pt:Fibroblasti:Qn:
Synonyms: Chimica Fibroblasto Miristato (C14:0) Palmitato (C16:0) Punto nel tempo (episodio) Rapporto di Concentrazione Catalitica
nl-NL Dutch (Netherlands) acyl-CoA-dehydrogenase.palmitaatsubstraat/acyl-CoA-dehydrogenasen.myristaatsubstraat:enzymatische activiteit verhouding:moment:fibroblast:kwantitatief:
tr-TR Turkish (Turkey) Açil KoA dehidrojenazlar.palmitat substrat/Açil KoA dehidrojeazlar.miristat substrat:KtlKonsOr:Zmlı:Fibroblastlar:Kant:
zh-CN Chinese (China) 酰基 CoA 脱氢酶.棕榈酸底物/酰基 CoA 脱氢酶.肉豆蔻酸底物:催化比率:时间点:成纤维细胞:定量型:
Synonyms: AcdA;酰基辅酶 A 脱氢酶 C14:0;十四酸;十四酸, C14:0;肉豆蔻酸, C14:0 C16:0;十六烷酸;十六烷酸, C16:0;十六酸;十六酸, C16:0;棕榈酸, C16:0;软脂酸;软脂酸, C16:0 催化比;催化比值;催化浓度比;催化浓度比值;催化浓度比率 化学;化学检验项目;化学检验项目类;化学类;化学试验;非刺激耐受型化学检验项目;非刺激耐受型化学检验项目类;非刺激耐受型化学试验;非刺激耐受型化学试验类 可用数量表示的;定量性;数值型;数量型;连续数值型标尺 时刻;随机;随意;瞬间 纤维原细胞;纤维母细胞 酰基 CoA 脱氢酶（AcdA、酰基辅酶 A 脱氢酶）.棕榈酸（C16:0、十六烷酸、十六烷酸, C16:0、十六酸、十六酸, C16:0、棕榈酸, C16:0、软脂酸、软脂酸, C16:0）底物 酰基 CoA 脱氢酶（AcdA、酰基辅酶 A 脱氢酶）.肉豆蔻酸（C14:0、十四酸、十四酸, C14:0、肉豆蔻酸, C14:0）底物

Example Units

Unit Source
{ratio} Example UCUM Units