506-46-7CCCCCCCCCCCCCCCCCCCCCCCCCC(=O)OXMHIUKTWLZUKEX-UHFFFAOYSA-NHexacosanoic acid is a 26-carbon, straight-chain, saturated fatty acid. It is a fatty acid 26:0, a very long-chain fatty acid and a straight-chain saturated fatty acid. It is a conjugate acid of a cerotate.
506-46-7DTXSID7075050CHEMBL464787CHEBI:31009Lipids -> Unambiguous Lipids
Fatty Acyls [FA] -> Fatty Acids and Conjugates [FA01] -> Straight chain fatty acids [FA0101]
White solid; [Acros Organics MSDS]
Solid
205.3 Ų [M-H]- [CCS Type: DT; Buffer gas: N2; Sample Type: Human bronchoalveolar lavage fluid (BALF); Dataset: Unambiguous Lipids]
201.57 Ų [M-H]- [CCS Type: TIMS; Method: single field calibrated]
안전 정보는 참고용이며, 공급업체의 물질안전보건자료(SDS), 관련 법규 및 전문가 평가를 대체하지 않습니다.
This chemical does not meet GHS hazard criteria for 66.7% (2 of 3) of all reports.
Warning
H315 (33.3%): Causes skin irritation [Warning Skin corrosion/irritation]
P261, P264, P264+P265, P271, P280, P302+P352, P304+P340, P305+P351+P338, P319, P321, P332+P317, P337+P317, P362+P364, P403+P233, P405, and P501 (click each P-code to see the statement)
Aggregated GHS information provided per 3 reports by companies from 2 notifications to the ECHA C&L Inventory. Each notification may be associated with multiple companies.
Chemical: Hexacosanoic acid
Hexacosanoic acid: Does not have an individual approval but may be used under an appropriate group standard
Treatment options for adrenoleukodystrophy (ALD) are limited. Dietary treatment is with Lorenzo's oil. For the childhood cerebral form, stem cell transplant and gene therapy are options if the disease is detected early in the clinical course. Adrenal insufficiency in ALD patients can be successfully treated. (Wikipedia)
Hexacosanoic acid, or cerotic acid, is associated with adrenoleukodystrophy (also known as X-linked adrenoleukodystrophy, ALD, X-ALD, adrenomyeloneuropathy, AMN, Siemerling–Creutzfeldt disease or bronze Schilder disease) is a disorder of peroxisomal fatty acid beta oxidation which results in the accumulation of very-long chain fatty acids in tissues throughout the body. The most severely affected tissues are the myelin in the central nervous system, the adrenal cortex and the Leydig cells in the testes. (Wikipedia)
용도Hexacosanoic acid may be used as a reference standard for the determination of hexacosanoic acid in human serum and plasma samples for the screening of peroxisomal disorders by gas chromatography-mass spectrometry (GC-MS) operating under selected-ion monitoring (SIM) modein biological samples by GC-MSin microalgal lipids by solid-phase extraction (SPE) followed by GC-MS analysisas a naphthoxyethyl derivative by high performance liquid chromatography (HPLC) coupled with fluorometric detection
일반 설명Hexacosanoic acid is classified under the very long chain fatty acids (VLCFAs) group of biologically important compounds.
불러오는 중…
white shiny fluffy or crystalline powder
Adrenoleukodystrophy (ALD) is caused by mutations in ABCD1, a gene located on the X chromosome that codes for ALD, a peroxisomal membrane transporter protein. The exact mechanism of the pathogenesis of the various forms of ALD is not known. Biochemically, individuals with ALD show very high levels of unbranched, saturated, very long chain fatty acids, particularly cerotic acid (26:0). The level of cerotic acid in plasma does not correlate with clinical presentation. (Wikipedia)
Clinically, adrenoleukodystrophy (ALD) is a heterogenous disorder, presenting with several distinct phenotypes, and no clear pattern of genotype-phenotype correlation. As an X-linked disorder, ALD presents most commonly in males, however approximately 50% of heterozygote females show some symptoms later in life. Approximately two-thirds of ALD patients will present with the childhood cerebral form of the disease, which is the most severe form. It is characterized by normal development in early childhood, followed by rapid degeneration to a vegetative state. The other forms of ALD vary in terms of onset and clinical severity, ranging from adrenal insufficiency to progressive paraparesis in early adulthood (this form of the disease is typically known as adrenomyeloneuropathy). (Wikipedia)
정의ChEBI: A 26-carbon, straight-chain, saturated fatty acid.