. Author manuscript; available in PMC: 2024 Feb 1.

Published in final edited form as: J Mol Cell Cardiol. 2019 Aug 13;135:109–118. doi: 10.1016/j.yjmcc.2019.08.005

Table 3.

Histopathological and molecular findings in HLHS.

Downregulated genes/proteins	Adrenergic receptors: β1-AR [87,88], Structural proteins: α-MHC, β-MHC [87], Calcium handling: SERCA 2a [87], Angiogenesis: VEGF [96]
Upregulated genes/proteins	Structural proteins: Collagen-1α, Collagen-3 (in non-failing Fontan) [124], β-MHC (in failing Fontan) [88], Transcription Factors: HIF-1α (marker of hypoxia) [96], p53 (marker of DNA damage) [96] Histones: ƔH2AX (marker of DNA damage) [96] Receptors: TGFβ1 (in fetal cardiomyocytes) [96] Adhesion molecules: PECAM-1 (inappropriately expressed in cardiomyocytes) [87]
Unchanged gene/protein expression	Adrenergic Receptors: β2-AR [87] Receptors: TGFβR1, TGFβR2 (markers of fibrosis) [124] Structural enzymes: MMP-2, MMP-9 [124]