Related Experiment Video
Updated: Aug 14, 2026
09:14
Improving 2D and 3D Skin In Vitro Models Using Macromolecular Crowding
Published on: August 22, 2016
Hair Follicle Microenvironment Changes in Collagen VI-Myopathy Patients with Alopecia
Francesca Pampaloni1,2, Bianca Maria Piraccini3, Francesca Bruni1,2
1Dermatology Units, IRCCS Azienda Ospedaliero-Università di Bologna, 40136 Bologna, Italy.
Cells
|August 13, 2026
Summary
Collagen VI-related myopathies (COL6-RM) can cause hair loss due to scalp and hair follicle changes. This study reveals inflammation and extracellular matrix remodeling in the hair follicle microenvironment of affected patients.
Area of Science:
- Dermatology
- Genetics
- Cell Biology
Background:
- Collagen VI-related myopathies (COL6-RM) are rare genetic disorders affecting muscle and skin.
- Alopecia (hair loss) is a newly identified scalp symptom in COL6-RM patients.
- The exact causes of hair follicle dysfunction in COL6-RM are not fully understood.
Purpose of the Study:
- To investigate the pathogenetic mechanisms of hair follicle dysfunction in COL6-RM.
- To characterize the scalp microenvironment in COL6-RM patients with alopecia.
Main Methods:
- Comprehensive immunohistochemical, ultrastructural, and proteomic analysis of scalp biopsies from six COL6-RM patients.
- Evaluation of hair follicle extracellular matrix and perifollicular adipose tissue.
- Proteomic profiling of adipokines and assessment of inflammatory markers.
Main Results:
- Structural changes were observed in the hair follicle's extracellular matrix, especially the inner fibrous sheath.
- Perifollicular adipose tissue showed enlarged lipid droplets, suggesting cell hypertrophy.
- Proteomic analysis revealed a pro-inflammatory and ECM-remodeling signature, with increased CD68 macrophages and MMP2 expression.
Conclusions:
- The findings indicate a complex pathological process involving extracellular matrix and adipose tissue remodeling in the hair follicle microenvironment.
- Collagen VI plays a potential role in regulating the hair follicle microenvironment in COL6-RM.
Related Concept Videos
01:05Type IV Collagen of Basal Lamina
Type IV collagen is a 400 nm long, network-forming collagen that acts as a barrier between the epithelial and endothelial cells. Type IV collagen forms the backbone of the basement membrane by scaffolding with laminin, entactin, proteoglycans, and fibronectin. Apart from rendering structural support to the basement membrane, it also helps entail signaling potentials necessary for both pathological and physiological functions.
A type IV collagen molecule has six alpha chains which can exist in...
A type IV collagen molecule has six alpha chains which can exist in...
01:11Fibril-associated Collagen
Fibril-associated collagens are a type of collagens present in the extracellular matrix with interrupted triple helices or FACIT (Fibril-associated collagens interrupted triple-helices). FACIT help connect and attach the collagen fibrils with each other as well as with other proteins of the extracellular matrix.
For example, the type II collagen fibrils in cartilage have covalently bound type IX fibril-associated collagens at regular intervals. Other types of fibril-associated collagens are...
For example, the type II collagen fibrils in cartilage have covalently bound type IX fibril-associated collagens at regular intervals. Other types of fibril-associated collagens are...
01:19The Effect of Aging on Tissues
Several body functions deteriorate with age. The external signs of aging are easily identifiable. For example, the skin becomes dry, less elastic, and thins out, forming wrinkles. The skin of the face begins to appear looser due to a decrease in the levels of elastic and collagen fibers in the connective tissue. Additionally, melanin production in the hair follicle decreases with age, resulting in gray hair. Moreover, the senses of sight and hearing decline, so glasses and hearing aids may...
01:28Phases of Wound Repair
Following injury, the integrity of the injured tissues must be reestablished. For example, in skin tissue, wound repair involves coordination among resident skin cells, blood mononuclear cells, extracellular matrix, growth factors, and cytokines to complete the healing cascade.
Formation of Blood Clot
In case of deep injuries, trauma to blood vessels results in blood loss. In the meantime, phospholipids released from the ruptured endothelial cellular membrane are converted into arachidonic...
Formation of Blood Clot
In case of deep injuries, trauma to blood vessels results in blood loss. In the meantime, phospholipids released from the ruptured endothelial cellular membrane are converted into arachidonic...
01:20Accessory Structures of the Skin: Hair Growth and Types
Hair growth begins with the production of keratinocytes by the basal cells of the hair bulb. As new cells are deposited at the hair bulb, the hair shaft is pushed through the follicle toward the surface. Keratinization is completed as the cells are pushed to the skin surface to form the shaft of hair that is externally visible. The external hair is completely dead and composed entirely of keratin. Hair can be cut or shaven without damaging the hair structure because the cut is superficial. Most...