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The finding that, as in humans, developmental programs in D. melanogaster require functional peroxisomes is demonstrated by the gross scale malformations noted in the organization of the central and peripheral nervous systems of mutant flies.
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Comparison of the hematopoietic system of compound mutant animals revealed numerous changes.
As a control experiment we also tested the olfactory system of the mutant mice.
In addition to altered functions in the central nervous system, the expression of mutant Htt was also found in peripheral tissues [ 2- 4], and was directly linked to local tissue defects [ 5, 6].
The present findings give further indication that the efferent systems of the bv/bv mutant inner ear are independent of the afferent systems in many aspects including development, maturation as well as degeneration.
The effects in experimental systems of the expression of mutant huntingtin, or a poly-glutamine n-terminal fragment, are diverse [10].
In pathological conditions, saturation of degradation systems by an excess of mutant proteins leads to their accumulation and aggregation.
The impaired leucine feedback system of these mutants resulted in increased 3H4MV fraction due to the increased metabolic flux to leucine biosynthesis.
These observations suggest that neural cell proliferation is increased in the nervous system of xav mutants.
In this study, we characterize the circadian system of mouse mutants lacking functional Sharp-1 and/or Sharp-2 genes.
Detailed histological analyses of the developing and mature nervous system of homozygous mutants did not reveal defects in overall brain size, patterning, cytoarchitecture or projections of major fibre tracts (Fig. S4 and data not shown).
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