ERAL1 Antibody

Code CSB-PA007759GA01HU
Size $600
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Product Details

Uniprot No.
Target Names
ERAL1
Alternative Names
CEGA antibody; Conserved ERA like GTPase antibody; Conserved ERA-like GTPase antibody; ERA antibody; Era (E. coli G protein homolog) like 1 antibody; Era G protein like 1 (E. coli) antibody; Era G protein like 1 antibody; ERA W antibody; ERA-like protein 1 antibody; ERA-W antibody; Eral1 antibody; ERAL1_HUMAN antibody; ERAL1A antibody; ERAW antibody; GTP binding protein era homolog antibody; GTPase Era antibody; GTPase Era; mitochondrial antibody; GTPase human homolog of E. coli essential cell cycle protein Era antibody; H-ERA antibody; HERA A antibody; hERA antibody; HERA B antibody; HERAA antibody; HERAB antibody; mitochondrial antibody
Raised in
Rabbit
Species Reactivity
Human,Mouse,Rat
Immunogen
Human ERAL1
Immunogen Species
Homo sapiens (Human)
Isotype
IgG
Purification Method
Antigen Affinity Purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Tested Applications
ELISA,WB,IHC
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

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.
Gene References into Functions
  1. Three women from three different families presented with signs of PS, including deafness and ovarian dysgenesis. We employed exome sequencing and identified a homozygous missense mutation in the ERAL1 gene, c.707A > T. The mutation leads to a damaging p.Asn236Ile substitution that is likely to interfere with the GTP binding capacity of the ERAL1 protein. PMID: 28449065
  2. Data show that loss of ERAL1 leads to rapid decay of nascent 12S mt-rRNA, consistent with a role as a mitochondrial RNA chaperone. PMID: 20604745
Involvement in disease
Perrault syndrome 6 (PRLTS6)
Subcellular Location
Mitochondrion matrix. Mitochondrion inner membrane; Peripheral membrane protein. Note=Localizes on the matrix side on the mitochondrial inner membrane.
Protein Families
TRAFAC class TrmE-Era-EngA-EngB-Septin-like GTPase superfamily, Era GTPase family
Database Links

HGNC: 3424

OMIM: 607435

KEGG: hsa:26284

STRING: 9606.ENSP00000254928

UniGene: Hs.3426

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7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA
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