ANTIBODIES FOR NEURO-RELATED DISEASES: ALS AND FTD

Frontotemporal lobar degeneration (FTD) and amyotrophic lateral sclerosis (ALS): a short tale of two neurodegenerative diseases.

Introduction

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.

Amyotrophic Lateral Sclerosis (ALS)

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 TDP43 (also known as TAR DNA-binding protein 43 or TARDBP), fused in sarcoma (FUS; Figure 1), ubiquilin2 (UBQLN2; Figure 2), SOD1 (Figure 3), and also in the non-coding region of C9orf72.

FUS mono antibody validated in iHC

Figure 1. Immunohistochemical staining of paraffin-embedded human gliomas tissue slide using 60160-1-Ig (FUS/TLS monoclonal antibody) at a dilution of 1:1000 (under 10x lens). A heat mediated antigen retrieval step was performed with Tris-EDTA buffer (pH9).

UBQLN2 antibody validated in WB

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.

 

SOD1 antibody validated in IHC

 

Figure 3. Immunohistochemical analysis of paraffin-embedded human testis using 10269-1-AP (SOD1 antibody) at dilution of 1:50 (under 10x lens). 

 

 

IHCeasy by proteintech is a ready-to-use IHC kit and it is the most complete IHC kit on the market. Primary antibody included along with other reagents.

 


 

Frontotemporal Dementia (FTD)

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.

C9ORF72 IN FTD AND ALS

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.

TDP43 antibody validated in IHC

Figure 4. Immunohistochemistry of paraffin-embedded mouse brain tissue slide using 10782-2-AP (TDP43 antibody) at a dilution of 1:200 (under 10x lens).

TAU antibody validated in IF

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).

 

Related Antibodies

Product Type Applications
TDP43 (C-Terminal) Antibody Rabbit polyclonal, KD/KO validated WB, IP, IHC, IF, chIP, ELISA
TDP43 (Human Specific) Antibody Mouse monoclonal WB, IP, IHC, FC, CoIP, ELISA
Recombinant TDP43 Antibody Rabbit recombinant, KD/KO validated WB, IHC, FC, ELISA
FUS/TLS Antibody Rabbit polyclonal, KD/KO validated WB, IP, IHC, IF, FC, chIP, ELISA
OPTN Antibody Rabbit polyclonal, KD/KO validated WB, IP, IHC, IF, FC, ELISA
GFAP Antibody Mouse monoclonal WB, IP, IHC, ELISA
PTEN Antibody Mouse monoclonal WB, IHC, IF, ELISA
C9orf72 Antibody Rabbit polyclonal, KD/KO validated WB, IP, IHC, IF, ELISA
VAPB Antibody Rabbit polyclonal, KD/KO validated WB, IP, IHC, IF, FC, ELISA
Ataxin 2 Antibody Rabbit polyclonal, KD/KO validated WB, IP, IHC, IF, ELISA
GR Repeat Antibody Rabbit polyclonal WB, IHC, IF, Dot blot, ELISA
GP Repeat Antibody Rabbit polyclonal WB, IHC, IF, ELISA
PR Repeat Antibody Rabbit polyclonal WB, IHC, IF, ELISA
P62/SQSTM1 Antibody Mouse monoclonal, KD/KO validated WB, IHC, IF, FC, ELISA
Granulin Antibody Rabbit polyclonal, KD/KO validated WB, IP, IHC, IF, ELISA
CHMP2B Antibody Rabbit polyclonal, KD/KO validated WB, IHC, IF, ELISA
Profilin 1 Antibody Rabbit polyclonal WB, IHC, IF, ELISA
VCP Antibody Rabbit polyclonal, KD/KO validated WB, IP, IHC, IF, CoIP, ELISA
GA Repeat Antibody Rabbit polyclonal WB, IHC, IF, ELISA
AP Repeat Antibody Rabbit polyclonal WB, IHC, IF, ELISA
TMEM106B Antibody Rabbit polyclonal WB, IP, IHC, IF, ELISA
Angiogenin Antibody Rabbit polyclonal WB, IHC, ELISA
P150 Glued Antibody Rabbit polyclonal WB, IHC, IF, ELISA
VGLUT1 Antibody Rabbit polyclonal IHC, IF, FC, ELISA
VCP Antibody Mouse monoclonal, KD/KO validated WB, IHC, IF, ELISA
ALS2 Antibody Rabbit polyclonal WB, IP, IHC, IF, ELISA
VGLUT2 Antibody Rabbit polyclonal WB, IHC, IF, ELISA

Final remarks

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. For example, recently, neuronal pentraxin 2 (NPTX2) has been identified as a reliable marker in f cerebrospinal fluid to monitor the progression of FTD. NPTX2 (10889-1-AP), also known as NARP (neuronal activity-regulated pentaxin), is secreted protein involved in excitatory synapse formation. It also plays a role in the clustering of alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA)-type glutamate receptors at established synapses, resulting in non-apoptotic cell death of dopaminergic nerve cells.