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Target Discovery and Validation Reviews and Protocols

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212<br />

Table 2<br />

Orthologous Human <strong>and</strong> Mouse Keratin Genes of Type II Cluster a<br />

Human Mouse Major Knockout Human<br />

keratin keratin expression phenotype pathology Reference<br />

K1 K1 n.d. Unknown<br />

K2 K2 n.d. Unknown<br />

K3 Unknown<br />

K4 K4 Stratified Cell fragility, acanthosis; White sponge Ness et al.<br />

epithelia K6 partially compensates nevus (1998) (Fig. 3D)<br />

K5 K5 Basal Neonatal death; skin Epidermolysis 21 (see Fig. 3A)<br />

epidermis blistering; cytolysis of bullosa simplex<br />

basal cells (EBS)<br />

K6a K6a Hair follicles; None; compensation Pachyonychia 96 (see Fig. 3E)<br />

activated by K6b congenita<br />

keratinocytes;<br />

nail bed<br />

epithelum<br />

K6a K6a Hair follicles; Tongue lesions; Pachyonychia 93,96 (Fig. 3E)<br />

K6b K6b activated postnatal death congenita<br />

keratinocytes; P10; Cell lysis<br />

nail bed upon migration<br />

epithelum during wound<br />

healing; 25% survival<br />

because of<br />

compensation by K18

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