The Paraplegin Polyclonal Antibody (CAB10249) is a valuable tool for researchers studying paraplegin, a protein associated with hereditary spastic paraplegia (HSP), a group of genetic disorders characterized by progressive weakness and stiffness in the legs. This polyclonal antibody, derived from rabbits, exhibits high specificity for human paraplegin and is validated for use in Western blot applications.Paraplegin is a mitochondrial protein involved in ATP-dependent proteolysis, playing a crucial role in mitochondrial quality control and cellular homeostasis. Dysfunctional paraplegin has been linked to neurodegenerative disorders such as HSP, making it a target of interest for therapeutic interventions aimed at preserving mitochondrial function.
By targeting paraplegin, researchers can gain insight into the molecular mechanisms underlying HSP and potentially develop new treatment strategies for this debilitating condition. The Paraplegin Polyclonal Antibody offers a reliable tool for detecting and analyzing paraplegin expression in various cell types, facilitating investigations into the pathophysiology of HSP and related disorders.
Product Name:
SPG7 Rabbit Polyclonal Antibody
SKU:
CAB10249
Size:
20uL, 100uL
Isotype:
IgG
Host Species:
Rabbit
Reactivity:
Human,Mouse,Rat
Immunogen:
Recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human SPG7 (NP_003110.1).
This gene encodes a mitochondrial metalloprotease protein that is a member of the AAA family. Members of this protein family share an ATPase domain and have roles in diverse cellular processes including membrane trafficking, intracellular motility, organelle biogenesis, protein folding, and proteolysis. Mutations in this gene cause autosomal recessive spastic paraplegia 7. Two transcript variants encoding distinct isoforms have been identified.
Purification Method:
Affinity purification
Gene ID:
6687
Storage Buffer:
Store at -20℃. Avoid freeze / thaw cycles.Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3.
Western blot analysis of various lysates using SPG7 Rabbit pAb (CAB10249) at 1:1000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution.Lysates / proteins: 25 μg per lane.Blocking buffer: 3 % nonfat dry milk in TBST.Detection: ECL Enhanced Kit (AbGn00021).Exposure time: 10s.