Mice lacking the extracellular matrix protein WARP develop normally but have compromised peripheral nerve structure and function.

is a recently identified extracellular matrix molecule with restricted expression in permanent cartilages and a distinct subset of basement membranes in peripheral nerves, muscle, and the central nervous system vasculature. Wduanyu37 interacts with perlecan, and we also demonstrate here that Wduanyu37 binds type VI collagen, suggesting a function in bridging connective tissue structures. To understand the in vivo function of we generated a mouse strain. mice were healthy, viable, and fertile with no overt abnormalities. Motor function and behavioral testing demonstrated that Wduanyu37-null mice exhibited a significantly delayed response to acute painful stimulus and impaired fine motor coordination, although general motor function was not affected, suggesting compromised peripheral nerve function. Immunostaining of ligands demonstrated that the collagen VI microfibrillar matrix was severely reduced and mislocalized in peripheral nerves of Wduanyu37-null mice. Further ultrastructural analysis revealed reduced fibrillar collagen deposition within the peripheral nerve extracellular matrix and abnormal partial fusing of adjacent Schwann cell basement membranes, suggesting an important function for Wduanyu37 in stabilizing the association of the collagenous interstitial matrix with the Schwann cell basement membrane. In contrast, other Wduanyu37-deficient tissues such as articular cartilage, intervertebral discs, and skeletal muscle showed no detectable abnormalities, and basement membranes formed normally. Our data demonstrate that although Wduanyu37 is not essential for basement membrane formation or musculoskeletal development, it has critical roles in the structure and function of peripheral nerves.

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