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Rabbit Anti-PAM16/FITC Conjugated antibody
background:
This gene encodes a mitochondrial protein involved in granulocyte-macrophage colony-stimulating factor (GM-CSF) signaling. This protein also plays a role in the import of nuclear-encoded mitochondrial proteins into the mitochondrial matrix and may be important in reactive oxygen species (ROS) homeostasis. Mutations in this gene cause Megarbane-Dagher-Melike type spondylometaphyseal dysplasia, an early lethal skeletal dysplasia characterized by short stature, developmental delay and other skeletal abnormalities. [provided by RefSeq, May 2017]
Function:
Regulates ATP-dependent protein translocation into the mitochondrial matrix. Inhibits DNAJC19 stimulation of HSPA9/Mortalin ATPase activity.
Subunit:
Probable component of the PAM complex at least composed of a mitochondrial HSP70 protein, GRPEL1 or GRPEL2, TIMM44, TIMM16/PAM16 and TIMM14/DNAJC19 (By similarity). Interacts with DNAJC19. Directly interacts with DNAJC15; this interaction counteracts DNAJC15-dependent stimulation of HSPA9 ATPase activity. Associates with the TIM23 complex.
Subcellular Location:
Mitochondrion inner membrane.
Tissue Specificity:
Ubiquitously expressed.
Similarity:
Belongs to the TIM16/PAM16 family.
Database links:
Entrez Gene: 51025 Human
Entrez Gene: 66449 Mouse
Omim: 614336 Human
SwissProt: Q9Y3D7 Human
SwissProt: Q9CQV1 Mouse
SwissProt: Q6EIX2 Rat
Unigene: 730693 Human
Unigene: 354760 Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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