Glycogen storage disease type Ⅰa(GSD-Ⅰa)is an autosomal recessive metabolic disorder caused by a deficiency in glucose-6-phosphatase-α(G6Pase-α or G6PC)that is expressed primarily in the liver,kidney,and intestine.G6Pase-α catalyzes the hydrolysis of glucose-6-phosphate(G6P)to glucose and phosphate in the terminal step of gluconeogenesis and glycogenolysis,and is a key enzyme for endog-enous glucose production.The active site of G6Pase-α is inside the endoplasmic reticulum(ER)lumen.For catalysis,the substrate G6P must be translocated from the cytoplasm into the ER lumen by a G6P transporter(G6PT).The functional coupling of G6Pase-α and G6PT maintains interprandial glucose ho-meostasis.Dietary therapies for GSD-Ⅰa are available,but cannot prevent the long-term complication of hepatocellular adenoma that may undergo malignant transformation to hepatocellular carcinoma.Ani-mal models of GSD-Ⅰa are now available and are being exploited to both delineate the disease more precisely and develop new treatment approaches,including gene therapy.

In maxillofacial surgery, there is a significant need for the design and fabrication of porous scaffolds with customizable bionic structures and mechanical properties suitable for bone tissue engineering. In this paper, we characterize the porous Ti6Al4V implant, which is one of the most promising and attractive biomedical applications due to the similarity of its modulus to human bones. We describe the mechanical properties of this implant, which we suggest is capable of providing important biological functions for bone tissue regeneration. We characterize a novel bionic design and fabrication process for porous implants. A design concept of "reducing dimensions and designing layer by layer" was used to construct layered slice and rod-connected mesh structure (LSRCMS) implants. Porous LSRCMS implants with different parameters and porosities were fabricated by selective laser melting (SLM). Printed samples were evaluated by microstructure characterization, specific mechanical properties were analyzed by mechanical tests, and finite element analysis was used to digitally calculate the stress characteristics of the LSRCMS under loading forces. Our results show that the samples fabricated by SLM had good structure printing quality with reasonable pore sizes. The porosity, pore size, and strut thickness of manufactured samples ranged from (60.95± 0.27)% to (81.23±0.32)%, (480±28) to (685±31) μm, and (263±28) to (265±28) μm, respectively. The compression results show that the Young's modulus and the yield strength ranged from (2.23±0.03) to (6.36±0.06) GPa and (21.36±0.42) to (122.85±3.85) MPa, respectively. We also show that the Young's modulus and yield strength of the LSRCMS samples can be predicted by the Gibson-Ashby model. Further, we prove the structural stability of our novel design by finite element analysis. Our results illustrate that our novel SLM-fabricated porous Ti6Al4V scaffolds based on an LSRCMS are a promising material for bone implants, and are potentially applicable to the field of bone defect repair.
Alloys ; Bionics ; Bone Substitutes/chemistry* ; Bone and Bones/pathology* ; Compressive Strength ; Elastic Modulus ; Finite Element Analysis ; Humans ; Lasers ; Materials Testing ; Maxillofacial Prosthesis Implantation ; Porosity ; Pressure ; Printing, Three-Dimensional ; Prostheses and Implants ; Prosthesis Design ; Stress, Mechanical ; Surgery, Oral/instrumentation* ; Tissue Engineering/methods* ; Titanium/chemistry*