345 Biomarkers Med. (2014) 8(3), 345–352 ISSN 1752-0363 part of Autism spectrum disorder (ASD) is a complex neurodevelopmental disorder characterized by impairments in reciprocal social interactions as well as restricted, repetitive and stereotyped patterns of behavior. The etiology of ASD is not well understood, although many factors have been associated with its pathogenesis, such genetic, neurological, environmental and immunological factors. Several studies have reported the production of numerous autoantibodies that react with specific brain proteins and brain tissues in autistic children and alter the function of the attacked brains tissue. In addition, the potential role of maternal autoantibodies to the fatal brain in the etiology of some cases of autism has also been reported. Identification and understanding of the role of brain autoantibodies as biological biomarkers may allow earlier detection of ASD, lead to a better understanding of the pathogenesis of ASD and have important therapeutic implications. Keywords:฀animal฀models฀•฀autism฀•฀autoimmunity฀•฀brain฀autoantibodies฀•฀BTBR mice •฀maternal฀antibodies฀•฀neurodevelopmental฀disorders Autism spectrum disorder (ASD) is a complex neurodevelopmental disorder characterized by impairments in reciprocal social interactions, such as verbal and nonverbal communication, and restricted, repetitive and stereotyped pat- terns of behavior. Recently, the prevalence of ASD has increased dramatically; the current prevalence of ASDs is estimated at 1 in 88 chil- dren, with a male to female ratio of approxi- mately 5:1 [1] . Patients are typically diagnosed before the age of 36 months [2] . The etiology and the clinical expression of the disorder is heterogeneous and complex [3,4]; multiple fac- tors have been suggested to be involved in its pathogenesis such as genetic, environmental, neurological and immunological factors [5–7] . There is strong evidence that genetic fac- tors such as HLADRB1*04 and complement component C4B can contribute to the patho- genesis of autism, as well as environmental fac- tors such as a viral infection that may cause autoimmunity to the brain and thereby may lead to pathological changes in the brain of autistic children [8] . The potential role of the immune system in ASD has been addressed in several studies, especially autoimmunity to CNS. It has been proposed that autoimmunity may play a key role in the pathogenesis of neurologic disorders, including ASD [2,9–12]. These are shown by several autoimmune factors: presence of brain- specific autoantibodies; impaired lymphocyte functions; abnormal cytokine regulation; viral associations; and indirect association of certain immunogenetic factors [13], as well as neuroglial activation and neuroinflammation in the CNS in autistic children [14]. In addition, a number of immune system-related genes have been linked to this disorder. These include the null allele of the C4B gene, a complement component, as well as the extended HLA [15–17]. The poten- tial role for maternal antibodies as a pathogenic factor has also been proposed [18–20]. Moreover, children with ASD were found to have a family history of autoimmune dis- orders, including asthma, multiple sclerosis (MS), rheumatoid arthritis, Type 2 diabetes mellitus and celiac disease [2,12,21] . Recently, Nadra E Elamin 1 & Laila Y AL-Ayadhi* ,1,2 1 Autism฀Research฀&฀Treatment฀Center,฀ Shaik฀AL-Amodi฀Autism฀Research฀ Chair,฀Faculty฀of฀Medicine,฀King฀Saud฀ University,฀Riyadh,฀Saudi฀Arabia 2 Department฀of฀Physiology,฀Faculty฀ of฀Medicine,฀King฀Saud฀University,฀ PO฀Box฀2925,฀Riyadh฀11461,฀Saudi฀Arabia *Author฀for฀correspondence: Tel.:฀+966฀11฀467฀1614 Fax:฀+966฀11฀462฀07207 ayadh2@gmail.com Brain autoantibodies in autism spectrum disorder Review 10.2217/BMM.14.1 © 2014 Future Medicine Ltd der