scholarly journals Unconventional secretion by autophagosome exocytosis

2010 ◽  
Vol 188 (4) ◽  
pp. 451-452 ◽  
Author(s):  
Suzanne R. Pfeffer
Keyword(s):  
Endoplasmic Reticulum ◽  
Coenzyme A ◽  
Signal Sequence ◽  
Binding Protein ◽  
Yeast Genetics ◽  
Medium Chain ◽  
Cell Biol ◽  
Chain Acyl ◽  
Unconventional Secretion ◽  
Acyl Coenzyme A

In this issue, Duran et al. (2010. J. Cell Biol. doi: 10.1083/jcb.200911154) and Manjithaya et al. (2010. J. Cell Biol. doi: 10.1083/jcb.200911149) use yeast genetics to reveal a role for autophagosome intermediates in the unconventional secretion of an acyl coenzyme A (CoA)–binding protein that lacks an endoplasmic reticulum signal sequence. Medium-chain acyl CoAs are also required and may be important for substrate routing to this pathway.

Interaction of acyl coenzyme A substrates and analogs with pig kidney medium-chain acyl-CoA dehydrogenase

Biochemistry ◽  
1987 ◽  
Vol 26 (12) ◽  
pp. 3704-3710 ◽  
Author(s):  
Patricia J. Powell ◽  
Sze Mei Lau ◽  
David Killian ◽  
Colin Thorpe
Keyword(s):  
Coenzyme A ◽  
Medium Chain ◽  
Pig Kidney ◽  
Chain Acyl ◽  
Acyl Coenzyme A

A fatal neonatal case of medium-chain acyl-coenzyme a dehydrogenase deficiency with homozygous A→G985 transition

1992 ◽  
Vol 121 (6) ◽  
pp. 965-968 ◽  
Author(s):  
Kin-Chuen Leung ◽  
Judith W. Hammond ◽  
Shilpi Chabra ◽  
Kevin H. Carpenter ◽  
Mary Potter ◽  
...  
Keyword(s):  
Dehydrogenase Deficiency ◽  
Coenzyme A ◽  
Medium Chain ◽  
Chain Acyl ◽  
Acyl Coenzyme A

Prevalence of medium-chain acyl-coenzyme A dehydrogenase deficiency in the sudden infant death syndrome

1993 ◽  
Vol 122 (5) ◽  
pp. 715-718 ◽  
Author(s):  
Raanan Arens ◽  
David Gozal ◽  
Karen Jain ◽  
Shamshad Muscati ◽  
Eva T. Heuser ◽  
...  
Keyword(s):  
Sudden Infant Death Syndrome ◽  
Infant Death ◽  
Dehydrogenase Deficiency ◽  
Coenzyme A ◽  
Sudden Infant Death ◽  
Sudden Infant ◽  
Medium Chain ◽  
Chain Acyl ◽  
Acyl Coenzyme A

Familial Reye-Like Syndrome: A Presentation of Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency

PEDIATRICS ◽  
1987 ◽  
Vol 79 (3) ◽  
pp. 382-385
Author(s):  
Bruce Taubman ◽  
Daniel E. Hale ◽  
Richard I. Kelley
Keyword(s):  
Dehydrogenase Deficiency ◽  
Coenzyme A ◽  
Term Therapy ◽  
Reye Syndrome ◽  
Medium Chain ◽  
Chain Acyl ◽  
History Of ◽  
Acyl Coenzyme A ◽  
Long Term Therapy

A 20-month-old girl with a family history of two siblings who died of an encephalopathy diagnosed as Reye syndrome presented to an emergency room in hypoglycemic coma and was found to have medium-chain acyl-coenzyme A dehydrogenase deficiency. The salient clinical and biochemical features of this newly described inborn error of fatty acid metabolism are described and contrasted to those of classical Reye syndrome. Important clues that should lead the clinician to suspect this disorder, methods of diagnosis, and appropriate acute and long-term therapy are also discussed.

Medium Chain Acyl–Coenzyme A Dehydrogenase Deficiency in a Neonate

2007 ◽  
Vol 357 (17) ◽  
pp. 1781-1781 ◽  
Author(s):  
Gregory Rice ◽  
Thomas Brazelton ◽  
Kathleen Maginot ◽  
Shardha Srinivasan ◽  
Gregory Hollman ◽  
...  
Keyword(s):  
Dehydrogenase Deficiency ◽  
Coenzyme A ◽  
Medium Chain ◽  
Chain Acyl ◽  
Acyl Coenzyme A

Gene delivery of medium chain acyl-coenzyme A dehydrogenase induces physiological cardiac hypertrophy and protects against pathological remodelling

2018 ◽  
Vol 132 (3) ◽  
pp. 381-397 ◽  
Author(s):  
Bianca C. Bernardo ◽  
Kate L. Weeks ◽  
Thawin Pongsukwechkul ◽  
Xiaoming Gao ◽  
Helen Kiriazis ◽  
...  
Keyword(s):  
Cardiac Hypertrophy ◽  
Coenzyme A ◽  
Healthy Adult ◽  
Capillary Density ◽  
Medium Chain ◽  
Adult Mice ◽  
Physiological Cardiac Hypertrophy ◽  
Chain Acyl ◽  
Acyl Coenzyme A

We previously showed that medium chain acyl-coenzyme A dehydrogenase (MCAD, key regulator of fatty acid oxidation) is positively modulated in the heart by the cardioprotective kinase, phosphoinositide 3-kinase (PI3K(p110α)). Disturbances in cardiac metabolism are a feature of heart failure (HF) patients and targeting metabolic defects is considered a potential therapeutic approach. The specific role of MCAD in the adult heart is unknown. To examine the role of MCAD in the heart and to assess the therapeutic potential of increasing MCAD in the failing heart, we developed a gene therapy tool using recombinant adeno-associated viral vectors (rAAV) encoding MCAD. We hypothesised that increasing MCAD expression may recapitulate the cardioprotective properties of PI3K(p110α). rAAV6:MCAD or rAAV6:control was delivered to healthy adult mice and to mice with pre-existing pathological hypertrophy and cardiac dysfunction due to transverse aortic constriction (TAC). In healthy mice, rAAV6:MCAD induced physiological hypertrophy (increase in heart size, normal systolic function and increased capillary density). In response to TAC (~15 weeks), heart weight/tibia length increased by ~60% in control mice and ~45% in rAAV6:MCAD mice compared with sham. This was associated with an increase in cardiomyocyte cross-sectional area in both TAC groups which was similar. However, hypertrophy in TAC rAAV6:MCAD mice was associated with less fibrosis, a trend for increased capillary density and a more favourable molecular profile compared with TAC rAAV6:control mice. In summary, MCAD induced physiological cardiac hypertrophy in healthy adult mice and attenuated features of pathological remodelling in a cardiac disease model.

First case report of medium-chain acyl-coenzyme A dehydrogenase deficiency in China

2015 ◽  
Vol 28 (5-6) ◽  
Author(s):  
CuiLi Liang ◽  
MinYan Jiang ◽  
HuiYing Sheng ◽  
YanNa Cai ◽  
DongYan Wu ◽  
...  
Keyword(s):  
Case Report ◽  
Inborn Error ◽  
Autosomal Recessive ◽  
Dehydrogenase Deficiency ◽  
Coenzyme A ◽  
Medium Chain ◽  
First Case ◽  
Mitochondrial Fatty Acid ◽  
Chain Acyl ◽  
Acyl Coenzyme A

AbstractMedium-chain acyl-coenzyme A dehydrogenase deficiency (MCADD) is an autosomal recessive inborn error of mitochondrial fatty acid β-oxidation, caused by mutations in the

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