Staffed by dedicated scientists with combined knowledge and expertise in complement therapeutic research, Creative Biolabs providing a full range of complement therapeutic services for our customer. Based on our extensive knowledge, we are fully equipped to reach out to our clients who may have problem or difficulty dealing with periodontal Ehlers-Danlos syndrome drug development.
Ehlers-Danlos Syndrome (EDS)
Named after two physicians at the turn of the 20th century, Ehlers-Danlos syndrome (EDS) is a group of heritable connective tissue disorder characterized by skin hyperextensibility, fragile and stretchy skin, unusual scars, easy bruising and generalized joint hypermobility. These could occur as early as at birth. EDS affects approximately 1 in 5,000 people all over the world. The prognosis depends on the specific type. In the majority of patients with classic EDS, the disease is caused by the mutation in one or more a dozen different genes. The specific gene determines the type of EDS. Thus, according to different mutations, EDS can be classified into 13 types as of 2017, with a significant overlap in features.
Periodontal Ehlers-Danlos syndrome (pEDS) is one subtype of EDS, which is transmitted in an autosomal dominant pattern and characterized by severe and intractable periodontitis of early childhood or adolescence, lack of attached gingiva, protibial plaques. In the teens, early-onset periodontitis leads to the inflammatory destruction of dental attachment and premature loss of teeth.
Features of pEDS
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Periodontitis
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Lack of attached gingiva
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Protibial plaques
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Pretibial hyperpigmentation
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Acrogeria
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Skin and gum fragility
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Dystrophic scarring
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Distal joint hypermobility
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Bruising out of proportion to trauma
Cause of pEDS
It is shown that pEDS is caused by mono-allelic missense or in-frame insertion/deletion alterations in C1R or C1S, the genes that encode the complement subunits C1r and C1s. These two proteins are sub-components of the C1 complex derived from the classical pathway of the complement system. Recognizing the antigen-antibody complex by the C1 complex initiates the classical pathway.
Diagnosis of pEDS
Genetic test: Genomic sequence analysis of C1r and C1s genes. Since it is discovered by scientists that pEDS is caused by the mutations of C1r and C1s genes derived from the complement system. Sequencing these two genes is an efficient way for the pEDS diagnosis.
Treatment of pEDS
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Mechanical debridement
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Adjunctive antibiotic therapy
With the rich research experiences in drug discovery and complement therapeutic field, Creative Biolabs' experts are confident in providing high-quality biotherapeutics development services based on the Complement Therapeutics platform. We offer turn-key or ala carte services customized to our client’s needs.
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Questions & Answer
A: The complement system, a key component of the innate immune system, is believed to be involved in the inflammatory processes associated with pEDS. While specific complement proteins and pathways have not been definitively identified, experimental research suggests that components of both the classical and alternative complement pathways may be activated in the context of pEDS. This is supported by findings of increased complement activation markers in pEDS patients' blood samples.
A: Experimental models for pEDS often include genetically engineered mice with COL3A1 mutations. These models help researchers understand the disease's mechanisms and test potential complement-targeted therapies.
A: Challenges include understanding the intricate complement system regulation role in pEDS and ensuring the safety and specificity of therapeutic interventions. Research is ongoing to optimize drug design and minimize off-target effects.
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