HSPE1 Antibody Pair

Definition and Function of HSPE1

HSPE1 encodes the 10 kDa mitochondrial chaperonin, a homolog of the bacterial GroES protein . It forms a heterodimer with HSPD1 (GroEL homolog) to facilitate protein folding in the mitochondrial matrix, particularly for oxidative phosphorylation (OXPHOS) complexes . Its expression is tightly regulated via a bidirectional promoter shared with HSPD1, ensuring coordinated transcription .

Key Functions:

• Assists in folding mitochondrial proteins (e.g., OXPHOS subunits) .

• Maintains mitochondrial proteostasis under stress conditions .

HSPE1 Antibody Pair: Product Details

The Abbexa HSPE1 Antibody Pair (catalog #abx500011) is a sandwich ELISA kit optimized for quantitative detection of HSPE1 in human, mouse, and guinea pig samples .

Performance Metrics:

• Sensitivity: Detects as low as 0.1 ng/mL of HSPE1 .

• Cross-reactivity: <1% with other heat shock proteins .

Mitochondrial Pathway Analysis

HSPE1 antibodies are used to study its role in oxidative phosphorylation. Co-expression analysis reveals HSPE1 interacts with OXPHOS proteins, while HSPD1 associates with TCA cycle enzymes . This modular organization underscores its specificity in mitochondrial pathways .

Disease-Associated Mutations

A de novo mutation in HSPE1 (p.Leu73Phe) was linked to infantile spasms, hypotonia, and mitochondrial dysfunction in a clinical case . Functional studies showed impaired HSP60/HSPE1 complex activity, highlighting the antibody’s utility in diagnosing chaperoninopathies .

Clinical Relevance and Disease Association

HSPE1 antibodies are critical for diagnosing mitochondrial disorders caused by chaperonin defects:

• Hereditary Spastic Paraplegia (SPG13): Mutations in HSPD1 disrupt mitochondrial protein folding .

• MitCHAP-60 Disease: Severe neurodegeneration due to HSPD1 mutations .

• HSPE1-Linked Disorders: Emerging evidence suggests HSPE1 mutations may contribute to early-onset mitochondrial diseases .