Cerebro-hepato-renal (Zellweger) syndrome and neonatal adrenoleukodystrophy: similarities in phenotype and accumulation of very long chain fatty acids.
about
The peroxisome: still a mysterious organelleNeonatal adrenoleukodystrophyPeroxisomal lignoceroyl-CoA ligase deficiency in childhood adrenoleukodystrophy and adrenomyeloneuropathy.The Δ4-desaturation pathway for DHA biosynthesis is operative in the human species: differences between normal controls and children with the Zellweger syndromeDefective peroxisomal cleavage of the C27-steroid side chain in the cerebro-hepato-renal syndrome of Zellweger.Platelet-activating factor: mediator of the third pathway of platelet aggregation? A study in three patients with deficient platelet-activating factor synthesisPeroxisomal beta-oxidation enzyme proteins in adrenoleukodystrophy: distinction between X-linked adrenoleukodystrophy and neonatal adrenoleukodystrophy.The significance of hyperpipecolatemia in Zellweger syndrome.A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase (pseudo-neonatal adrenoleukodystrophy)Catalase deficiency accelerates diabetic renal injury through peroxisomal dysfunctionUltrastructural and cytochemical demonstration of peroxisomes in cultured fibroblasts from patients with peroxisomal deficiency disorders.Lignoceric acid is oxidized in the peroxisome: implications for the Zellweger cerebro-hepato-renal syndrome and adrenoleukodystrophy.Teratogenic inborn errors of metabolism.Biosynthesis and maturation of peroxisomal beta-oxidation enzymes in fibroblasts in relation to the Zellweger syndrome and infantile Refsum diseasePeroxisomal organization in normal and cerebrohepatorenal (Zellweger) syndrome fibroblasts.Peroxisomes: a nexus for lipid metabolism and cellular signaling.Organelle pathology in metabolic neuromuscular disease: an overview.Multiple peroxisomal enzymatic deficiency disorders. A comparative biochemical and morphologic study of Zellweger cerebrohepatorenal syndrome and neonatal adrenoleukodystrophyMetabolism of saturated and polyunsaturated fatty acids by normal and Zellweger syndrome skin fibroblasts.Structure and lipid distribution of polyenoic very-long-chain fatty acids in the brain of peroxisome-deficient patients (Zellweger syndrome).Leopard spot retinal pigmentation in infancy indicating a peroxisomal disorder.Retinal energy demands control vascular supply of the retina in development and disease: The role of neuronal lipid and glucose metabolism.The peroxisome: an update on mysteries 2.0
P2860
Q28754736-5E092870-DE5F-4F89-B7A7-22813D63B261Q33621848-F68F0C7A-72AB-46ED-8DF3-AB1AEE5E6020Q33655555-BB598B77-5D3C-44DE-A4C5-7829168B5D58Q33688532-3CB522D9-054D-4118-8AA6-FA8FD646B850Q34529995-0F2802C7-C035-4EDB-A868-48E1A1E33130Q34557708-F70CB20F-F9F8-401B-A254-E730DA183758Q34602966-387159C2-FE80-4D71-87DA-CC54B74CB323Q35200897-810B1AEF-5900-4129-AEF4-159BFD43D05EQ35245375-8ADAE465-91D9-40D2-B525-7C95CF50E49CQ35766906-E0E08EC8-29D6-4B09-ADB0-227AE6A210EBQ36213312-E46AAB5B-CE55-43B4-8A86-040F9D4B345DQ36265198-65D55C75-24A9-435B-B0F3-D885C85F1297Q36706948-AFC6B65A-7F23-4F79-8D2D-79C54B65EDD2Q37395755-9A560F8C-05C3-4B8A-BEBF-F278051E89FBQ37550462-E4BACCB1-A6BF-4CCB-AB58-A021165BFADCQ37633445-A0BECF4C-AAC0-4E1A-8ED9-F15274571306Q38145146-C72BD2B1-5CCB-4908-8551-F19DA319A411Q41806915-0F84B1EB-52EB-4BB2-A645-A903991F2011Q42050577-D16478E3-A5E2-4CDE-9C53-4D7EE62C91BBQ42857172-5680023A-DC29-4C6F-9A49-F1D541F1FD2EQ43008088-18F0FA24-BB42-4F7B-A437-8B7466AA6B52Q49632281-8F155FDA-772D-4CAC-988D-B3CA49A4251CQ57921401-0F4AD8D1-E3EC-4C6E-B4C7-5372BAF9FC9E
P2860
Cerebro-hepato-renal (Zellweger) syndrome and neonatal adrenoleukodystrophy: similarities in phenotype and accumulation of very long chain fatty acids.
description
1982 nî lūn-bûn
@nan
1982年の論文
@ja
1982年学术文章
@wuu
1982年学术文章
@zh-cn
1982年学术文章
@zh-hans
1982年学术文章
@zh-my
1982年学术文章
@zh-sg
1982年學術文章
@yue
1982年學術文章
@zh
1982年學術文章
@zh-hant
name
Cerebro-hepato-renal (Zellwege ...... f very long chain fatty acids.
@en
Cerebro-hepato-renal
@nl
type
label
Cerebro-hepato-renal (Zellwege ...... f very long chain fatty acids.
@en
Cerebro-hepato-renal
@nl
prefLabel
Cerebro-hepato-renal (Zellwege ...... f very long chain fatty acids.
@en
Cerebro-hepato-renal
@nl
P2093
P1476
Cerebro-hepato-renal (Zellwege ...... of very long chain fatty acids
@en
P2093
Brown FR 3rd
Cummins JW
McAdams AJ
P304
P407
P577
1982-12-01T00:00:00Z