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The only form that occurs via autosomal\u2010dominant inheritance exhibits adult onset and is sometimes referred to as Parry type NCL. The manifestations may include behavioral symptoms followed by seizures, ataxia, dementia, and early death. Mutations in the gene\n                    <jats:italic>DNAJC5<\/jats:italic>\n                    that codes for the presynaptic co\u2010chaperone cysteine string protein\u2010\u03b1 (CSP\u03b1) were recently reported in sporadic adult\u2010onset cases and in families with dominant inheritance. The mutant CSP\u03b1 protein may lead to disease progression by both loss and gain of function mechanisms. Iron chelation therapy may be considered as a possible pharmaceutical intervention based on our recent mechanism\u2010based proposal of CSP\u03b1 oligomerization via ectopic Fe\u2010S cluster\u2010binding, summarized in this review.\n                  <\/jats:p>","DOI":"10.1111\/cge.13829","type":"journal-article","created":{"date-parts":[[2020,8,12]],"date-time":"2020-08-12T03:41:42Z","timestamp":1597203702000},"page":"111-118","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":34,"title":["Autosomal dominant neuronal ceroid lipofuscinosis: Clinical features and molecular basis"],"prefix":"10.1111","volume":"99","author":[{"given":"Nima","family":"Naseri","sequence":"first","affiliation":[{"name":"Appel Institute for Alzheimer's Disease Research, and Brain &amp; Mind Research Institute Weill Cornell Medicine  New York City New York 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