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Function

Catalyzes the attachment of leucine to its cognate tRNA.

Involvement in disease

Perrault syndrome 4

PRLTS4

An autosomal recessive, sex-influenced disorder characterized by sensorineural deafness in both males and females, and ovarian dysgenesis in females. Affected females have primary amenorrhea, streak gonads, and infertility, whereas affected males show normal pubertal development and are fertile.

None

The disease is caused by variants affecting the gene represented in this entry.

Hydrops, lactic acidosis, and sideroblastic anemia

HLASA

A lethal, multisystem metabolic disorder characterized by severe lactic acidosis, hydrops, and sideroblastic anemia. Additional features include impaired cardiac function, disordered coagulation, pulmonary hypertension, and progressive renal disease.

None

The disease is caused by variants affecting the gene represented in this entry.

Sequence similarities

Belongs to the class-I aminoacyl-tRNA synthetase family.

Tissue specificity

Ubiquitously expressed, but highest expression in tissues with high metabolic rates, such as skeletal muscle, heart, and kidney.

Cellular localization

  • Mitochondrion matrix

Alternative names

KIAA0028, LARS2, Leucyl-tRNA synthetase, LeuRS

Target type

Proteins

Molecular weight

101976Da

We found 1 product in 1 category

Proteins & Peptides

Target

Species of origin

Search our catalogue for 'LARS2' (1)

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