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Frontotemporal lobar degeneration (FTD) and amyotrophic lateral sclerosis (ALS): a short tale of two neurodegenerative diseases.
Neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), Alzheimer’s disease (AD), frontotemporal dementia (FTD), Huntington’s disease (HD), Parkinson’s disease (PD), spinocerebellar ataxia (SCA), and spinal muscular atrophies (SMAs), are incurable and debilitating disorders.
ALS (also known as Lou Gehrig’s disease) is a common adult-onset neuropathological disease. Typical symptoms include hand and leg weakness, loss of ability to initiate/control movements, and slurred speech. This disorder is marked by the loss of motor neurons in the brain and spinal cord, and atrophy of the frontal and temporal lobes.
The majority of pathogenic mutations are present in TAR DNA-binding protein 43 (TARDBP/TDP-43), fused in sarcoma (FUS; Figure 1), ubiquilin2 (UBQLN2; Figure 2), SOD1 (Figure 3), and also in the non-coding region of C9orf72.
Figure 1. Immunohistochemical staining of paraffin-embedded human gliomas tissue slide using 60160-1-Ig (FUS/TLS antibody) at a dilution of 1:1000 (under 10x lens). A heat mediated antigen retrieval step was performed with Tris-EDTA buffer (pH9).
Figure 2. Rat brain tissue lysate was subjected to SDS PAGE followed by western blot with 23449-1-AP (UBQLN2 antibody) at a dilution of 1:800 incubated at room temperature for 1.5 hours.
Figure 3. Immunohistochemical staining of paraffin-embedded human brain using 10269-1-AP (SOD1 antibody) at a dilution of 1:50 (under 10x lens).
|Ataxin 2||P150 Glued||VGLUT1|
FTD is a disease caused by progressive nerve cell loss. The majority of cases have brain atrophy in the frontal and/or temporal lobes. FTD is known as young-onset dementia and is characterized by behavioral abnormalities including a decline in cognitive, motor, and language function.
Most common FTD mutations are related to the TARDBP/TDP-43 protein (Figure 4), TAU (Figure 5) pathology, or the fused in sarcoma (FUS) gene.
ALS and FTD have been classified as belonging to the same family spectrum diseases, where hexanucleotide repeat expansions (HRE) of GGGGCC in C9orf72 is a key genetic cause player. Recent studies by Davidson Y et al. have confirmed the high specificity of Proteintech C9orf72 antibodies, highlighting their great value for research and diagnostic purposes.
Figure 4. Immunohistochemistry of paraffin-embedded mouse brain tissue slide using 10782-2-AP (TDP-43 antibody) at a dilution of 1:200 (under 10x lens).
Figure 5. Immunofluorescent analysis of (4% PFA) fixed mouse brain tissue using 10274-1-AP (TAU antibody) at a dilution of 1:50 and Alexa Fluor 488-conjugated AffiniPure Goat Anti-Rabbit IgG(H+L).
|AP Repeat||GP Repeat|
|C9orf72||GR Repeat||TDP-43 (C-Terminal)|
|GA Repeat||PR Repeat||VCP|
New drugs should be developed to have neuroprotection properties via the improvement of synaptic plasticity, reduced oxidant and inflammation damage, or rescue mobility dysfunctions.
With an increasingly aging global population, the economic as well as human impact of neurodegenerative disorders is expected to increase unless reliable tools and care strategies can be developed.