ERAL1 Recombinant Monoclonal Antibody

Code CSB-RA007759MA1HU
Size US$210
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Image
  • IHC image of CSB-RA007759MA1HU diluted at 1:200 and staining in paraffin-embedded human small intestine tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-human polymer IgG labeled by HRP and visualized using 0.05% DAB.
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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
Species Reactivity
Human
Immunogen
Recombinant Human ERAL1 protein
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated
Clonality
Monoclonal
Isotype
hIgG1
Clone No.
27A9
Purification Method
Affinity-chromatography
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Form
Liquid
Tested Applications
ELISA, IHC
Recommended Dilution
Application Recommended Dilution
IHC 1:50-1:200
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.
Usage
For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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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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