The Virtuoso, The Chromosome, and The Devil

(NOTE: You can play the video and continue to scroll)

The 6th Chromosome in humans, unfortunately, shares a numerical identity with what a few religions consider to be the Devil. It is however, a rather inconspicuous part of the 48 chromosomes that make up the human genome. It contributes to around 6% of the total DNA in each cell of our body. If it could speak, it would attempt to distance itself from this unfortunate association by drawing attention to the fact that the most famous of the genes it contains are the ones encoding the Major Histocompatibility Complex or MHC, which enables the cells of our immune system to differentiate between self and foreign cells or proteins; an extremely vital component of our immunity. In the year 1782 however, a tiny aberration in this chromosome would make an entire hall of people come face to face with the devil.

A caricature of Paganini by Cesare Bacchi, 1940]

In the 1800s, music lovers would recall the concerts they attended of an 18 year old virtuoso. He was abnormally tall and thin. Attention would be drawn to his large black eyes, hooked nose, and jet black hair that hung limply his shoulders. His most striking feature however, a morbid pallor, like one risen from the grave; thrown into greater contrast by his hair and choice of dark clothing. As the audience looked on with unease, the youth would take the stage and begin his performance. Any doubts the audience harbored solidified into a collective certainty.

Dramatisation of Paganini playing his 24th Caprice in the movie The Devil’s Violinist

Niccolo Paganini, violin perched on his shoulder, would produce music the likes of which was not just unheard of, but was thought of as impossible to produce on the violin. His fingers would fly deftly across the strings, extending and moving at unnatural angles and speeds. Jumping between octaves the practiced ease of a cat jumping at a mouse, in front of half a thousand spectators, one of his strings would snap. He would continue playing unconcernedly; reaching further and further down the remaining strings to play. A second string would snap (perhaps as planned), then a third and he would keep playing, bending the single string to his will to act as all four. At the end of the performance audiences would remark that they SAW the devil himself pull the bow as he used both his hands to finger the strings.  Ludwig Rellstab, famed German poet and music critic remarked, “Never in my whole life have I heard an instrument weep like this…  I never knew that music contained such sounds! He spoke, he wept, he sang! There is something demonic about him. Goethe’s Mephisto would have played the violin like this.” (Sic. Courcy 1957, 1:318).

Paganini himself, did nothing to refute these rumors, in fact he actively tried to lend them greater credulence by composing more and more outlandish (for the time) music and dressing and behaving in a way as to suggest that there really was a demonic aspect to him. At one concert in Nice, he was required to procure a letter signed by his mother that he wasn’t the result of her liaisons with the Devil.  This title he would acquire of “The Demon Violinist” would follow him to his grave.

Niccolo Paganini may have been the most famous case of such a “devil” but he was part of a legacy spanning back to 400 BC, finding its first mention in Hippocrates’ Air, Water and Fire. A legacy that was first brought into the purview of modern medicine in the year 1657 when the Dutch surgeon Job Janszoon van Meek’ren encountered a sailor by the name of Georg Albes who displayed an extreme hyperextensibility of his skin. He could “stretch the skin of his chin, reaching his chest, or covering his eyes, and the skin over his knees could be pulled out to the length of half an ell.”

The first complete description of this phenomenon would be given in the year 1892 by Dr. Tschernogobov at the Moscow Dermatology and Venereology Society as a syndrome displaying laxity and fragility of the skin associated with hypermobility of the large joints.

It was this hypermobility of his fingers that allowed Paganini to play at unprecedented skill levels on the violin. He is said to have been able to extend his thumb so far back that his nail could touch the back of his hand, the flexibility of his wrist and fingers allowing him to span up to 3 octaves on the violin with little effort. Other individuals with similar features displayed their hyper flexibility in the 19th century, performing in travelling shows with titles such as “The Elastic Lady” or “The India Rubber Man.”

This syndrome gained formal recognition in the 20th century when Edvard Lauritz Ehlers of Denmark and Henri-Alexandre Danlos of France separately provided case descriptions of similar patients in 1901 and 1908 respectively; immortalizing their names in the field of Medicine in the form of Ehlers-Danlos Syndrome.

Our understanding of this disease grew in leaps and bounds in the next hundred years and an abnormality of Collagen was determined to be the basic pathology underlying the condition.

Collagen is the most abundant protein present in mammals, forming the primary fibrous component of skin, bone, tendon, cartilage and teeth. Classical Ehlers-Danlos Syndrome (EDS) Type I and II are caused to the defective pos-translational modification of collagen i.e. the hydroxylation of proline and lysine residues within fibroblasts.

Above: Clinical signs of EDS; Below: Electron Microscopic view of collagen deposition: Normal (left) vs EDS (right).

The more common form of EDS, which Paganini is suspected to have suffered from, is Type III which has its origins in the TNXB gene on Chromosome 6.

The TNXB gene is peculiar in the fact that it overlaps the Cyp21B gene which codes for the steroid, 21-hydroxylase. The haploinsufficiency of TNXB was found to be responsible for a new type of EDS, first noted in patients suffering from Congenital Adrenal Hyperplasia. It was inherited in a recessive fashion and did not show the phenotype of atrophic scarring, a hallmark of classical EDS, and was labeled as Benign Joint Hypermobility Syndrome, later classified as Ehlers-Danlos Syndrome Type III.

The TNXB gene was found to code for the protein Tenascin-X. The deficiency of this protein leads to the loss of collagen density as well as orientation throughout the body, despite the collagen itself being normal. This disorganized collagen accounts for some of the most common manifestations of EDS Type III – easy bruising, skin laxity, and – the devil’s gift to Paganini – joint laxity. This gift however, was a double edged sword.

Microscopic view of dermis showing Collagen I and Fibronectin structure in Normal (wildtype) and TNXB negative knockout mice

Though the myth behind Paganini’s deal with the Devil may have been just that – a myth – his condition did resemble a bad bargain in the sense that it came at a price.

The fated mutation in the 6th chromosome of Paganini’s genome, in addition to contributing to his mastery of the violin, also led to a plethora of manifestations which plagued the virtuoso for most of his life. The EDS led him to suffer from chronic gastric discomfort and constipation since a young age for which he was prescribed Mercury (Calomel), according to the prevailing practice in the day, to which he developed an addiction and consumed for most of his life leading to manifestations of mercury poisoning such as irritability, avoidance behavior and lassitude. It also led to the development of a postural tremor in his later years which would cause him great difficulty in writing and playing the violin.

The mysterious persona that Paganini had created around himself, coupled with his incredible performances and appearance, made him one of the first “rockstars” in the world of music. He drew large crowds of adoring fans which included a rather dedicated and enthusiastic following of ladies in particular, compounded by the fact that Paganini was known to be a serial womanizer himself. Due to these circumstances, his earlier symptoms along with a gradually progressive dysphonia, were attributed to Syphilis, one of the common diseases of the era. The treatment of choice for Syphilis at that time? Mercury.

His health declined further due to the emergence of a persistent hemoptysis, attributed initially to tuberculosis, a diagnosis which was subsequently discarded as his lungs were normal on examination. This too was most likely a manifestation of EDS die to increased capillary fragility. He died on May 27th, 1840 in Nice. The fame woven around his persona, the legend of his association with the Devil, led to the Bishop refusing permission for his burial, his body remained in a basement till the 1876.

Though the Bishop of Nice may have tried to deny Paganini his eternal rest, his actions had, unbeknownst to him, granted him a form of immortality. His unique style of music would continue to inspire composers and performers for generations. The original rockstar in the world of music, he had forged the culture of associating similar actions and modern music with the devil.

Over the period of a century or more, the “riffs” he played with unmatched technical proficiency on the violin, helped in no small degree by his 6th chromosome, found an echo in the 6 strings of the guitar. The ‘tapping’ guitar solos popularized by Van Halen can be considered the spiritual successor of the unique style of pseudo-tapping pizzicato (at 2:32 in the first video) Paganini worked into his 24th Caprice. Perhaps the most enduring legacy of Paganini is in the field of Neo Classical Metal, a genre combining the elements of blues rock and metal with the technical aspects and melodies of classical musical. Yngwie Malmsteen, one of the pioneers of this genre was heavily influenced by the works of Paganini among others.

Yngwie Malsteem plays Paganini’s 4th Concerto

The origin of a new style of music, the birth of rockstar culture, and the demonization of a violinist, can all be traced back to that one fateful moment of mutation of one segment on the short arm of the 6th chromosome of the son of an unsuccessful Italian trader. What caused this mutation? Was just one of the million mutations that can occur at any given point of time in anyone? Or was it the work – of the Devil.


Author

Kshitij Nair

Stubborn introvert who was borderline forced to write this description.
Doesn’t know how to handle criticism nor praise.
Secretly craves both.

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