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. 2018 Jun;188(6):1419–1429. doi: 10.1016/j.ajpath.2018.02.008

Figure 2.

Figure 2

Functional abnormalities in NIPA-like domain–containing 4 (NIPAL4) deficiency reflect cytotoxicity, with impaired lipid secretion. A and B: Elevated transepidermal water loss (TEWL) and reduced stratum corneum (SC) hydration (by corneometry) in affected canines. C and F: Representative images show reduced lamellar contents at the stratum granulosum (SG)–SC interface (open arrows), with appearance of prominent nonlamellar domains (asterisks) (C, postfixation ruthenium tetroxide). D and E: Decreased or patchy Nile red lipid staining in frozen sections from NIPAL4-deficient (open arrows) versus normal canine (arrows). F and H: Stripped membranes, with positive acid lipase labeling (arrows) of cytosolic organelles in stratum spinosum (SS) and SG of affected [ichthyin (Ich)] canines and abnormal lamellar body structure (open arrows) (postfixation osmium tetroxide). G: Pyroantimonate cytochemistry identifies Ca++ ion deposits within stripped membranes (postfixation osmium tetroxide). Scale bars: 0.1 μm (C); 200 μm (D and E); 2 μm (F and H); 0.2 μm (G). AU, absolute units.