2008
DOI: 10.1242/dev.012377
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KBP is essential for axonal structure, outgrowth and maintenance in zebrafish, providing insight into the cellular basis of Goldberg-Shprintzen syndrome

Abstract: Mutations in Kif1-binding protein/KIAA1279 (KBP) cause the devastating neurological disorder Goldberg-Shprintzen syndrome (GSS) in humans. The cellular function of KBP and the basis of the symptoms of GSS, however, remain unclear. Here, we report the identification and characterization of a zebrafish kbp mutant. We show that kbp is required for axonal outgrowth and maintenance. In vivo time-lapse analysis of neuronal development shows that the speed of early axonal outgrowth is reduced in both the peripheral a… Show more

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Cited by 86 publications
(88 citation statements)
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“…KBP was originally identified as a KIF1-binding protein (Wozniak et al, 2005), and its zebrafish kbp mutants have defective axonal outgrowth and maintenance (Lyons et al, 2008). Furthermore, mutations in the human KBP/KIAA1279 gene are associated with Goldberg-Shprintzen syndrome, a disorder characterized by malfunction of the central and enteric nervous systems (Brooks et al, 2005).…”
Section: Discussionmentioning
confidence: 99%
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“…KBP was originally identified as a KIF1-binding protein (Wozniak et al, 2005), and its zebrafish kbp mutants have defective axonal outgrowth and maintenance (Lyons et al, 2008). Furthermore, mutations in the human KBP/KIAA1279 gene are associated with Goldberg-Shprintzen syndrome, a disorder characterized by malfunction of the central and enteric nervous systems (Brooks et al, 2005).…”
Section: Discussionmentioning
confidence: 99%
“…Another possibility is that the motor domainless forms inhibit the functions of both kinesins by competing for the common associated proteins. KBP was recently identified as a KIF1-binding protein associated with mitochondria, and its zebrafish kbp mutants exhibit a defect in the distribution of axonal mitochondria (Wozniak et al, 2005;Lyons et al, 2008). Therefore, we examined the interaction between KLP6 and KBP.…”
Section: Three Kinesins Participate In Mitochondrial Movementsmentioning
confidence: 99%
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“…Preparation of tissue was as carried out as described previously (Lyons et al, 2008). Microwave stimulation during tissue processing was carried out using a Panasonic microwave with 'Inverter' technology.…”
Section: Transmission Electron Microscopymentioning
confidence: 99%
“…Zebrafish kbp was shown to be required for axonal outgrowth in the central and peripheral nervous system. In the kbp mutant fish, axonal microtubules were disrupted indicating neuronal cytoskeletal defects as a cause for GOSHS (Lyons et al 2008). In addition to KIF1B, KBP binds other proteins associated with microtubules, including several kinesin motor proteins and the microtubule destabilizing protein SCG10 (Alves et al 2010, Lehti et al 2013.…”
Section: Introductionmentioning
confidence: 99%