GTPase Era, mitochondrial

Definition:

Probable GTPase that plays a role in the mitochondrial ribosomal small subunit assembly. Specifically binds the 12S mitochondrial rRNA (12S mt-rRNA) to a 33 nucleotide section delineating the 3' terminal stem-loop region. May act as a chaperone that protects the 12S mt-rRNA on the 28S mitoribosomal subunit during ribosomal small subunit assembly.

References:

[1]. Sven Dennerlein, et al. Human ERAL1 is a mitochondrial RNA chaperone involved in the assembly of the 28S small mitochondrial ribosomal subunit. Biochem J. 2010 Sep 15;430(3):551-8. [Content Brief]

[2]. Iliana A Chatzispyrou, et al. A homozygous missense mutation in ERAL1, encoding a mitochondrial rRNA chaperone, causes Perrault syndrome. Hum Mol Genet. 2017 Jul 1;26(13):2541-2550. [Content Brief]

[3]. Takeshi Uchiumi, et al. ERAL1 is associated with mitochondrial ribosome and elimination of ERAL1 leads to mitochondrial dysfunction and growth retardation. Nucleic Acids Res. 2010 Sep;38(16):5554-68. [Content Brief]

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