Disease relevance of Zfp259

• These data identify ZPR1 deficiency as a contributing factor in neurodegenerative disorders [1].
• Spinal muscular atrophy disrupts the interaction of ZPR1 with the SMN protein [2].

High impact information on Zfp259

• Deletion analysis demonstrated that this binding interaction is mediated by the zinc fingers of ZPR1 and subdomains X and XI of the EGFR tyrosine kinase [3].
• The effect of EGF to regulate ZPR1 binding is dependent on tyrosine phosphorylation of the EGFR [3].
• One potential modifier gene is represented by ZPR1, which is down-regulated in patients with SMA and encodes a zinc finger protein that interacts with complexes formed by SMN [1].
• To test the functional significance of ZPR1 gene down-regulation, we examined a mouse model with targeted ablation of the Zpr1 gene [1].
• We found that Zpr1-/- mice die during early embryonic development, with reduced proliferation and increased apoptosis [4].

Biological context of Zfp259

• Together, these data indicate that ZPR1 contributes to the regulation of SMN complexes and that it is essential for cell survival [4].
• These results suggest that a high beef tallow diet up-regulates ZPR1 mRNA expression in the brain and might increase the vulnerability to oxidative stress [5].
• The increased ZPR1 expression in the neuronal, Neuro-2A cells, caused a significant increase in H(2)O(2)-induced cell death [5].

Anatomical context of Zfp259

• ZPR1 is essential for survival and is required for localization of the survival motor neurons (SMN) protein to Cajal bodies [4].
• These effects of Zpr1 gene disruption were confirmed and extended in studies of cultured motor neuron-like cells using small interfering RNA-mediated Zpr1 gene suppression; ZPR1 deficiency caused growth cone retraction, axonal defects, and apoptosis [4].
• Expression of ZPR1 mRNA in the cerebellum and hippocampus was elevated in response to the high beef tallow diet [5].

Analytical, diagnostic and therapeutic context of Zfp259

• The zinc finger protein ZPR1 translocates from the cytoplasm to the nucleus after treatment of cells with mitogens [6].

References