
@article {117970,
	title = {Neurological Consequences of Sphingosine Phosphate Lyase Insufficiency.},
	journal = {Front Cell Neurosci},
	volume = {16},
	year = {2022},
	month = {2022},
	pages = {938693},
	abstract = {<p>In 2017, an inborn error of metabolism caused by recessive mutations in was discovered. The disease features steroid-resistant nephrotic syndrome, adrenal insufficiency, and neurological defects. The latter can include sensorineural hearing loss, cranial nerve defects, peripheral neuropathy, abnormal brain development, seizures and/or neurodegeneration. encodes the pyridoxal-5{\textquoteright}-phosphate (PLP) dependent enzyme sphingosine phosphate lyase (SPL), and the condition is now referred to as SPL insufficiency syndrome (SPLIS). SPL catalyzes the final step in the degradative pathway of sphingolipids in which the bioactive sphingolipid sphingosine-1-phosphate (S1P) is irreversibly degraded to a long chain aldehyde and phosphoethanolamine (PE). SPL guards the only exit point for sphingolipid metabolism, and its inactivation leads to accumulation of various types of sphingolipids which have biophysical roles in plasma membrane rafts and myelin, and signaling roles in cell cycle progression, vesicular trafficking, cell migration, and programmed cell death. In addition, the products of the SPL reaction have biological functions including regulation of autophagic flux, which is important in axonal and neuronal integrity. In this review, the neurological manifestations of SPLIS will be described, and insights regarding the neurological consequences of SPL insufficiency from the study of brain-specific SPL knockout mice and SPL mutants will be summarized.</p>
},
	issn = {1662-5102},
	doi = {10.3389/fncel.2022.938693},
	author = {Atreya, Krishan B and Saba, Julie D}
}
@article {62775,
	title = {Adult Drosophila muscle morphometry through microCT reveals dynamics during ageing.},
	journal = {Open Biol},
	volume = {9},
	year = {2019},
	month = {2019 Jun 28},
	pages = {190087},
	abstract = {<p>Indirect flight muscles (IFMs) in adult Drosophila provide the key power stroke for wing beating. They also serve as a valuable model for studying muscle development. An age-dependent decline in Drosophila free flight has been documented, but its relation to gross muscle structure has not yet been explored satisfactorily. Such analyses are impeded by conventional histological preparations and imaging techniques that limit exact morphometry of flight muscles. In this study, we employ microCT scanning on a tissue preparation that retains muscle morphology under homeostatic conditions. Focusing on a subset of IFMs called the dorsal longitudinal muscles (DLMs), we find that DLM volumes increase with age, partially due to the increased separation between myofibrillar fascicles, in a sex-dependent manner. We have uncovered and quantified asymmetry in the size of these muscles on either side of the longitudinal midline. Measurements of this resolution and scale make substantive studies that test the connection between form and function possible. We also demonstrate the application of this method to other insect species making it a valuable tool for histological analysis of insect biodiversity.</p>
},
	issn = {2046-2441},
	doi = {10.1098/rsob.190087},
	author = {Chaturvedi, Dhananjay and Prabhakar, Sunil and Aggarwal, Aman and Atreya, Krishan B and VijayRaghavan, K}
}
