UBE3A Antibodies

Structure of UBE3A

UBE3A is a ubiquitin ligase with a molecular weight of approximately 100 kDa. Its precise molecular weight may fluctuate slightly due to differences in transcript subtypes and amino acid sequences among species.

This protein is composed of approximately 865 amino acids, and its spatial structure mainly consists of the receptor binding region at the N-terminal and the HECT catalytic domain at the C-terminal. The HECT domain is the core for its E3 ubiquitin ligase activity, capable of binding to ubiquitin and directly transferring it to the target protein. Its tertiary structure forms a unique L-shaped conformation, which is crucial for identifying specific substrates and completing ubiquitination modifications. The cysteine residue located at the catalytic center is responsible for forming thioester bonds with ubiquitin, while the adjacent multiple cyclic structures work together in coordination to ensure the specificity and efficiency of ubiquitin transfer.

Fig. 1 Schematic representation of polyubiquitination process by UBE3A ligase containing HECT domain.¹

Key structural properties of UBE3A:

• Contains characteristic HECT domains
• Form a unique L-shaped three-dimensional conformation
• The catalytic center contains conserved cysteine residues
• Multiple functional circular areas work in synergy

Functions of UBE3A

The core function of the UBE3A protein is to act as an E3 ubiquitin ligase to regulate substrate degradation. In addition, it is also involved in various neurophysiological processes such as neuronal development and synaptic plasticity.

The functional loss of UBE3A is closely related to Angelman syndrome. Its enzyme activity has high substrate specificity, which is directly related to its unique HECT domain conformation, making it play an irreplaceable regulatory role in the nervous system.

Applications of UBE3A and UBE3A Antibody in Literature

1. Vatsa, Naman, and Nihar Ranjan Jana. "UBE3A and its link with autism." Frontiers in molecular neuroscience 11 (2018): 448. https://doi.org/10.3389/fnmol.2018.00448

The article indicates that the UBE3A gene is imprinted and expressed in the brain, and it has the functions of both ubiquitin ligase and transcriptional coactivator. Its activity must be precisely regulated: maternal genetic deletion leads to Angelman syndrome, while repetition or functional enhancement is associated with autism, highlighting its core role in the regulation of synaptic function.

2. Vihma, Hanna, et al. "Ube3a unsilencer for the potential treatment of Angelman syndrome." Nature Communications 15.1 (2024): 5558. https://doi.org/10.1038/s41467-024-49788-8

The article indicates that due to the paternal UBE3A being silenced by non-coding RNA (UBE3A-ATS) in neurons, the deletion of the maternal allele can trigger Angelman syndrome. Research has found that the small molecule compound (S)-PHA533533 can effectively down-regulate UBE3A-TS and reactivate the paternal UBE3A through a new mechanism, bringing hope for non-invasive treatment of this disease.

3. Roy, Bidisha, et al. "UBE3A: the role in autism spectrum disorders (ASDs) and a potential candidate for biomarker studies and designing therapeutic strategies." Diseases 12.1 (2023): 7. https://doi.org/10.3390/diseases12010007

The article indicates that the UBE3A gene is a key candidate gene for autism spectrum disorder (ASD) in the q11-q13 region of chromosome 15. This gene is imprinted and expressed in the brain. Its function-acquired mutations, duplications or triploids are all associated with various ASDs such as Angelman syndrome. This review aims to deeply explore the association mechanism between UBE3A and ASD and look forward to its prospects as a diagnostic biomarker and therapeutic target.

4. Chaudhary, Pankaj, Jessica Proulx, and In-Woo Park. "Ubiquitin-protein ligase E3A (UBE3A) mediation of viral infection and human diseases." Virus Research 335 (2023): 199191. https://doi.org/10.1016/j.virusres.2023.199191

The article indicates that UBE3A (also known as E6-AP) is a multifunctional ubiquitin ligase that not only regulates protein degradation but also functions as a transcriptional coactivator. This gene mutation can lead to neurodevelopmental disorders such as Angelman syndrome and autism, and it also profoundly affects the stability of viral proteins and the progression of viral diseases. This review aims to explore the molecular mechanism by which UBE3A is involved in disease formation and viral regulation.

5. Yang, Xin, and Yu-Wen Alvin Huang. "Unraveling the Roles of UBE3A in Neurodevelopment and Neurodegeneration." International Journal of Molecular Sciences 26.5 (2025): 2304. https://doi.org/10.3390/ijms26052304

The article indicates that as a key ubiquitin ligase, the abnormal function of UBE3A is closely related to autism, Angelman syndrome and various neurodegenerative diseases. This review not only Outlines the expression of UBE3A in neurons and glial cells, but also proposes an innovative bidirectional interaction model, aiming to provide new ideas for the treatment of brain diseases targeting UBE3A.

Creative Biolabs: UBE3A Antibodies for Research

Creative Biolabs specializes in the production of high-quality UBE3A antibodies for research and industrial applications. Our portfolio includes monoclonal antibodies tailored for ELISA, Flow Cytometry, Western blot, immunohistochemistry, and other diagnostic methodologies.

• Custom UBE3A Antibody Development: Tailor-made solutions to meet specific research requirements.
• Bulk Production: Large-scale antibody manufacturing for industry partners.
• Technical Support: Expert consultation for protocol optimization and troubleshooting.
• Aliquoting Services: Conveniently sized aliquots for long-term storage and consistent experimental outcomes.

For more details on our UBE3A antibodies, custom preparations, or technical support, contact us at email.