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42623

Sigma-Aldrich

Butyryl-L-carnitine

≥97.0% (TLC)

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Synonym(s):
(2R)-3-Carboxy-N,N,N-trimethyl-2-(1-oxobutoxy)-1-propanaminium inner salt, Butanoyl-L-carnitine, C4-Carnitine
Empirical Formula (Hill Notation):
C11H21NO4
CAS Number:
Molecular Weight:
231.29
MDL number:
PubChem Substance ID:
NACRES:
NA.26

Quality Level

assay

≥97.0% (TLC)

form

powder, crystals or chunks

optical activity

[α]/D -23±2°, c = 1 in H2O

color

white to off-white

storage temp.

2-8°C

SMILES string

C[N+](C)(C)C[C@H](OC(CCC)=O)CC([O-])=O

InChI

1S/C11H21NO4/c1-5-6-11(15)16-9(7-10(13)14)8-12(2,3)4/h9H,5-8H2,1-4H3/t9-/m1/s1

InChI key

QWYFHHGCZUCMBN-SECBINFHSA-N

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1 of 4

This Item
504055108551371
Butyryl-L-carnitine ≥97.0% (TLC)

42623

Butyryl-L-carnitine

2-Methylbutyryl-L-carnitine ≥97.0% (HPLC)

50405

2-Methylbutyryl-L-carnitine

Isobutyryl-L-carnitine ≥97.0% (TLC)

51085

Isobutyryl-L-carnitine

Isovaleryl-L-carnitine ≥94.0% (HPLC)

51371

Isovaleryl-L-carnitine

assay

≥97.0% (TLC)

assay

≥97.0% (HPLC)

assay

≥97.0% (TLC)

assay

≥94.0% (HPLC)

storage temp.

2-8°C

storage temp.

2-8°C

storage temp.

2-8°C

storage temp.

2-8°C

optical activity

[α]/D -23±2°, c = 1 in H2O

optical activity

[α]/D -20±2°, c = 0.1 in H2O

optical activity

[α]/D -22±2°, c = 1 in H2O

optical activity

[α]/D -23±2°, c = 1 in H2O

Quality Level

100

Quality Level

100

Quality Level

100

Quality Level

100

color

white to off-white

color

white to off-white

color

white

color

white to off-white

Biochem/physiol Actions

Butyrylcarnitine is elevated in patients with Acyl-CoA dehydrogenase, short-chain (SCAD) deficiency, in infants with acute acidosis and generalized muscle weakness and in middle-aged patients with chronic myopathy localized in muscle; very long chain Acyl-CoA dehydrogenase deficiency, and celiac disease.

Storage Class

11 - Combustible Solids

wgk_germany

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable


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Sarah P Young et al.
Clinica chimica acta; international journal of clinical chemistry, 337(1-2), 103-113 (2003-10-22)
Homozygosity and compound heterozygosity for the short chain acyl-CoA dehydrogenase (SCAD) gene sequence variants 625G-->A and 511C-->T are associated with ethylmalonic aciduria (EMA), a biochemical indicator of SCAD deficiency. The clinical and biochemical implications of these variants are not fully
R Golan et al.
International journal of andrology, 6(4), 349-357 (1983-08-01)
Bioautography of human semen demonstrated the presence of L-carnitine, acetylcarnitine, propionylcarnitine and C4-acylcarnitines (butyrylcarnitines). In studies designed to ascertain the organs secreting these compounds into semen it was found that: Quantitative analyses of semen obtained pre- and post-vasectomy showed markedly
S Benito et al.
The Analyst, 143(18), 4448-4458 (2018-08-29)
Pediatric chronic kidney disease (CKD) is a clinical syndrome characterized by renal hypofunction occurring due to gradual and irreversible kidney damage that can further progress over time. New biomarkers may help early diagnosis of pediatric patients suffering from CKD and
A Bhala et al.
The Journal of pediatrics, 126(6), 910-915 (1995-06-01)
We identified two additional patients with short-chain acyl-coenzyme A (CoA), further characterized the clinical and biochemical features of this defect, and compared it with other fatty acid oxidation defects. We have measured the in vitro short-chain acyl-coenzyme A dehydrogenase (SCAD)
Josiane Joseph et al.
Metabolites, 8(4) (2018-10-05)
Duchenne muscular dystrophy (DMD) is a musculoskeletal disorder that causes severe morbidity and reduced lifespan. Individuals with DMD have an X-linked mutation that impairs their ability to produce functional dystrophin protein in muscle. No cure exists for this disease and

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