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. 2023 Mar 24;14:1092205. doi: 10.3389/fneur.2023.1092205

Table 2.

Main proteins identified in the ependymal intercellular junctions.

Tight junctions Adherens junctions Gap junctions Associated proteins
Ependymal junctional proteins by age
Embryonic Ependyma Inline graphic Claudin-5 (E17) (14) N-cadherin (E17) (14)
Protocadherin γ-C3 (E17) (14)
T-cadherin (E17) (14)		 β-catenin 1 (AJ) (E17) (14)
β-catenin 2 (AJ) (E17) (14)
α-catenin 1 (AJ) (E17) (14)
β-catenin-interacting protein 1 (AJ) (E17) (14)
δ-catenin-2 (AJ) (E17) (14)
Adult Ependyma Inline graphic Claudin-11 (14)
Claudin-1 (22)
Claudin-2 (22)
Claudin-3 (22)

Occludin

(52) Jam-2 (14)
Jam-3
Subependymal calcifications, hemorrhagic destruction of the brain, hydrocephalus (15).

ZO-1 (20)		 Plakophilin-4 (14)
N-cadherin
Hydrocephalus, ependymal denudation, SBA, deficiency of Cx43 and intracellular trafficking (24–28).

E-cadherin
Protocadherin γ-C3 (14)		 Connexin-29 (14)
Connexin-30 (57)
Connexin-32 (14)
Connexin-43 (58)
Connexin-26 (spinal ependyma) (112)		 MDPZ (TJ)
Congenital hydrocephalus with ependymal hydrocephalus (17–19).
β-catenin 1 (AJ) (14)
α-catenin 1 (AJ) (14)
δ-catenin-2 (AJ) (14)
α-catenin 2 (AJ) (14) Dlg5 (AJ)
Failure of t-SNARE dependent vesicular trafficking, loss of catenin-cadherin adhesion and cellular orientation (29).
αSNAP (AJ)
Failure of vesicular trafficking and loss of E-cadherin (38).
IIIG9 (AJ)
Deletion causes hydrocephalus and ependymal denudation (33).
Nonmuscle Myosin II-B (AJ) Hydrocephalus and ependymal denudation (36).

Proteins are divided by developmental age (embryonal vs. adult) and function. The main clinical correlates are listed for each junctional element. A schematic of the ependymal changes during embryonic and adult life is provided in the left column. AJ, adherens junction; TJ, tight junction.