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Unlike the retina in the homozygous (rds−/−) mouse, which fails to form OSs and undergoes fairly rapid apoptotic photoreceptor cell death, the retina in the heterozygous (rds+/−) mouse exhibits a classic, well-defined adRP phenotype characterized by early onset rod degeneration and late onset cone degeneration.
Considering the relative roles of IR versus hyperinsulinemia in dyslipidemia, Kahn discussed analyses of the LIRKO mouse, which fails to express hepatic insulin receptors, making the insulin level irrelevant (18).
Christopher Niyibizi (Hershey Medical School, Hershey, PA, USA) studied the gene therapy of OI using the oim mouse, which fails to produce the α2-chain of type I collagen and has a recessive condition resembling human OI.
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Moreover, phenotypic correction assayed by tail clip challenge resulted in survival of all AAV1-F9 treanimalsinals, in contrast to naive mice and 50% of AAV2-treated hemophilia B mice, which failed to survive.
This observation is in accordance with the previous descriptions of other Card15/Nod2 KO models of mice which failed to reveal gross intestinal abnormalities under basal conditions [7], [25].
Briefly, human neonatal foreskin was engrafted onto the hind flanks of C.B.-17 scid mice, which fail to reject the xenografts.
This is in stark contrast to the FVB Db VP2 mice which failed to generate an antibody response to recombinant VP2.
This abnormal distribution is similar to that described in Caspr1-null mice, which fail to generate calyceal junctions (Sousa et al., 2009).
Studies in PDX-1 deficient mice (which fail to develop G-cells) demonstrated no alteration in gastric amylin expression, confirming predominant expression of amylin within D-cells [ 23].
The importance of M-CSF-induced osteoclastogenesis is confirmed in mouse models: op/op mice, which fail to express functional M-CSF, and c-fms (the M-CSF receptor) deficient mice show an osteoclast-poor osteopetrotic phenotype [ 99, 100].
Using p47-/ mice, which fail to produce oxygen radicals via NADPH oxidase, we have shown that during IFN-γ-stimulated IC-mediated arthritis, oxygen radicals completely determine chondrocyte death and aggravate MMP-mediated cartilage destruction.
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