Recombinant Human GTPase Era, mitochondrial (ERAL1)

Uniprot Id

O75616

Target Species

Human

Target Name

ERAL1

Target Full Name

GTPase Era, mitochondrial

Target Function

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.

Target Involvement

Perrault syndrome 6 (PRLTS6)

Target Subcellular Location

Mitochondrion matrix. Mitochondrion inner membrane; Peripheral membrane protein. Note=Localizes on the matrix side on the mitochondrial inner membrane.

Target Protein Families

TRAFAC class TrmE-Era-EngA-EngB-Septin-like GTPase superfamily, Era GTPase family

Target Synonyms

CEGA; Conserved ERA like GTPase; Conserved ERA-like GTPase; ERA ; Era (E. coli G protein homolog) like 1; Era G protein like 1 (E. coli) ; Era G protein like 1; ERA W; ERA-like protein 1; ERA-W; Eral1; ERAL1_HUMAN; ERAL1A; ERAW; GTP binding protein era homolog; GTPase Era; GTPase Era; mitochondrial; GTPase human homolog of E. coli essential cell cycle protein Era ; H-ERA; HERA A; hERA; HERA B; HERAA; HERAB; mitochondrial

Target Background

The protein encoded by this gene is a GTPase that localizes to the mitochondrion. The encoded protein binds to the 3' terminal stem loop of 12S mitochondrial rRNA and is required for proper assembly of the 28S small mitochondrial ribosomal subunit. Deletion of this gene has been shown to cause mitochondrial dysfunction, growth retardation, and apoptosis. Several transcript variants encoding different isoforms have been found for this gene.