HSPD1

HSPD1

A gene on chromosome 2q33.1 that encodes a mitochondrial protein of the chaperonin family, which may function as a signalling molecule in the innate immune system. It is essential for the folding and assembly of newly imported proteins in mitochondria. HSPD1 is adjacent to a related gene; the region between the two acts as a bidirectional promoter.

Molecular pathology
HSPD1 mutations cause autosomal recessive spastic paraplegia 13.
References in periodicals archive ?
Es una proteina codificada por el gen HSPD1 (posicion 2q33.1), compuesta por 573 aminoacidos lo cual corresponde a un peso molecular de 61,05 kDa (31).
Embryos cultured at atmospheric oxygen tension (20% [O.sub.2]) also presented higher relative abundance of HSP90AA1, HSPD1, and MORF4L2 genes related to response to cellular stress and DNA damage repair.
Human beta cells also expressed 2-times more heat shock 60 kD (HSPD1), 10 kD (HSPE1), and 27/28 kD (HSPB1) (Figure 4(a)).
Our data also exhibited that the stress-inducible protein-1 (STPI1) regulated HSP90AB1 and HSPD1. STIP1 is an adaptor protein that coordinates the functions of HSP70 and HSP90 in protein folding.
As shown in Figure 5, leucine treatment markedly increased the mitochondrial component genes expression (Figure 5(a), dark columns), Hspd1 (P = 0.003) and COX2 (P = 0.003).
De otro lado, en funcion del consumo de energia, se consideran dos grupos, las dependientes de ATP, como Hsp100/HSPH, Hsp90/HSPC1, Hsp70/HSPA, Hsp60/ HSPD1, Hsp40/DNAJB1 y las independientes de ATP como Hsp/HSPB (13).
The two genes, hspd1 and mps1, had been found in other studies to be required for fin and heart regeneration in zebrafish, and Qin's work showed that they also were switched on in Muller glia from damaged zebrafish retinas.
Fang and his team studied the role of miRNA-382 in an obstructed kidney and reported that the abundance of miRNA-382 was associated with silencing of heat shock protein 60 (HSPD1) along with upregulation of 3-nitrotyrosine (3-NT) and downregulation of thioredoxin (Trx).
Murine antihuman Hsp60 Abs were from Santa Cruz (Heidelberg, Germany; clones H-1, H-300, K-19, LK1), BD Biosciences (San Diego, CA, USA; clone 24/HSP60), Novus (Littleton, CO, USA; clone Mab11-13), Lifespan (Seattle, WA, USA; clone HSPD1), and Thermo Scientific (Rockford, IL, USA; clone 4B9/89).
Indeed, heat shock protein family A (Hsp70) member 4 (HSPA4) was significantly underexpressed upon HS treatment and, on the contrary, heat shock protein family A (Hsp70) member 9 (HSPA9), heat shock protein family D (Hsp60) member 1 (HSPD1), and heat shock protein family E (Hsp10) member 1 (HSPE1) were slightly overexpressed.
Proteomic and bioinformatic analyses revealed that heat shock protein 60 (HSPD60, HSPD1) was a target gene of miR-382 [5].
Also another form of HSP, that is, SPG13, is caused by mutations in the HSPD1 gene that encodes heat shock protein 60, which is crucial for the folding of several mitochondrial proteins, once again affecting mitochondrial function [102].