Name: ERAL1 (docaravimab Biosimilar) Recombinant Monoclonal Antibody
Brand: CUSABIO
SKU: CSB-RA007759MB1HU

Product Details

Uniprot No.

O75616

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

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

0.01M PBS，pH7.4

Form

Liquid

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Notes

Validation Status
Application-specific performance (e.g., in flow cytometry, ELISA, IHC or other assay formats) has not yet been experimentally verified by CUSABIO. Users are advised to determine the optimal working conditions empirically in their own assay systems.
Guaranteed Quality
① Antibody purity > 95% tested by SDS-PAGE.
② Endotoxin level < 0.1EU/ug tested by LAL method.

Lead Time

In stock

Description

This recombinant monoclonal antibody is developed as a research-grade biosimilar to docaravimab, targeting ERAL1 (Era-like 12S mitochondrial rRNA chaperone 1). ERAL1 is a mitochondrial GTPase that plays a critical role in mitochondrial ribosome biogenesis, specifically functioning as an RNA chaperone essential for the maturation of the 12S mitochondrial ribosomal RNA. This protein is vital for proper mitochondrial translation and oxidative phosphorylation. Mutations in ERAL1 have been associated with severe mitochondrial disorders, including Perrault syndrome, characterized by sensorineural hearing loss and ovarian dysfunction, as well as other mitochondrial encephalopathies presenting with developmental delays and neurological impairment.

As a biosimilar to docaravimab, this antibody provides researchers with a reliable tool for investigating ERAL1 expression, localization, and function in mitochondrial biology studies. It enables exploration of mitochondrial ribosome assembly mechanisms, mitochondrial disease pathogenesis, and potential therapeutic targets for inherited mitochondrial disorders. This reagent supports investigations into cellular energy metabolism and mitochondrial dysfunction across various experimental contexts.

Usage

It is a non-therapeutic biosimilar antibody, owning the same variable region from the corresponding approved therapeutic antibody. In conclusion, it is a research-grade biosimilar antibody and expressed in mammalian cell, which can be directly used as positive controls in drug discovery or used for rapid verification of the biological functions of target protein.

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

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
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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Code	CSB-RA007759MB1HU
Size	US$9799
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ERAL1 (docaravimab Biosimilar) Recombinant Monoclonal Antibody

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Target Background