| Gene Symbol | SACSPROTEIN |
| Protein Type | Chaperone |
| Function | is a 4,579-amino acid multidomain protein that integrates chaperone activity with mitochondrial regulation and cytoskeletal remodeling<sup>[1]</sup>. |
| Subcellular Localization | ** | Cytoplasm, mitochondria-associated | |
| Molecular Weight | 520 kDa |
| Amino Acids | 579 aa |
| GeneCards | SACSPROTEIN |
| Human Protein Atlas | SACSPROTEIN |
| Three Sacsin Repeating Regions (SRR1-3) | Hsp90-like ATPase domains; each ~400 aa with ATP binding and hydrolysis |
| XPCB domain | Xeroderma pigmentosum group C binding domain; involved in DNA damage response |
| DnaJ domain | J-domain that recruits and activates Hsp70 chaperones |
| Fission promotion | Loss of sacsin causes hyperfused, elongated mitochondrial networks |
| Mitophagy | Required for efficient PINK1/Parkin-mediated mitophagy |
| Bioenergetics | Sacsin-deficient cells show reduced mitochondrial membrane potential and ATP production |
| Calcium buffering | Mitochondrial elongation impairs Ca²⁺ uptake dynamics |
| Associated Diseases | neurodegeneration |
| Databases | GeneCardsUniProtNCBI GeneHPASTRING |